Materiały źródłowe

• #1 POEMS Syndrome: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1097031-overview

  POEMS syndrome is rare, with several hundred cases described in the literature; however, the incidence may be underreported because the syndrome may go unrecognized. […] The onset of POEMS syndrome occurs most frequently in the fifth or sixth decade of life (mean age at onset, 48 y for men and 59 y for women). In 2007 and 2008, however, POEMS syndrome was reported to occur in two 15-year old patients. […] POEMS syndrome is slightly more prevalent among men than women, with a male-to-female ratio of 2.5:1. No specific racial association has been identified, though a preponderance of cases have been reported in the Japanese literature.

• #2 Recent Advances in the Treatment and Supportive Care of POEMS Syndrome

  https://www.mdpi.com/2077-0383/11/23/7011

  POEMS is a rare clonal plasma cell disorder characterized by multi-systemic features that include demyelinating peripheral neuropathy, organomegaly, endocrinopathy, presence of monoclonal proteins (M-protein), and skin changes. […] The diagnosis of POEMS is not always straight forward and often the diagnosis is delayed. It is based on fulfilling mandatory criteria of polyradiculoneuropathy and monoclonal protein and the presence of one major criterion (Castleman disease, sclerotic bone lesions, or elevated VEGF), and at least one minor criterion. […] Due to the rarity of the syndrome, clear guidelines for treatment are still lacking. […] The serum VEGF level is elevated in most patients with POEMS. […] The probability to have POEMS gets higher with greater serum VEFG levels. […] The serum VEGF is markedly elevated and correlates with the activity of the disease.

• #3 Prevalence, clinical profiles, and prognosis of POEMS syndrome in Japanese nationwide survey – PubMed

  https://pubmed.ncbi.nlm.nih.gov/31371568/

  Objective: To elucidate current epidemiological, clinical profiles, and treatment of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome. […] We conducted a nationwide survey in 2015 using an established epidemiologic method. Data processing sheets were sent to all neurology and hematology specialist departments throughout Japan to identify patients with POEMS who were seen between April 2012 and March 2015. […] The estimated number of patients with POEMS was 392 (95% confidence interval [CI] 320-464), and the prevalence was 0.3 per 100,000. […] This study showed current epidemiologic and clinical status of POEMS syndrome in Japan.

• #4 Orphanet: POEMS syndrome

  https://www.orpha.net/en/disease/detail/2905

  POEMS syndrome is a paraneoplastic syndrome characterized by polyradiculoneuropathy (P), organomegaly (O), endocrinopathy (E), clonal plasma cell disorder (M), and skin changes (S). […] Exact prevalence and incidence rates are not known. The only estimates are from Japan. In this country, prevalence has been reported to be about 1/330,000. Male predilection has been reported (2.5:1). […] The average age of onset is in the 5th or 6th decade. […] The main differential diagnoses are chronic inflammatory demyelinating polyneuropathy, AL amyloidosis and Guillain-Barr syndrome. Monoclonal gammopathy of undetermined significance (MGUS) and should also be considered.

• #5 POEMS syndrome epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/POEMS_syndrome_epidemiology_and_demographics

  The precise incidence of POEMS syndrome is difficult to determine due to the complexity of the syndrome presentation and we have no well established data regarding the incidence. […] Prevalence of POEMS syndrome in Japan is reported to be about 0.3 per 100,000 individuals. […] The median age at presentation is 51 years. […] There is no racial predilection to POEMS syndrome. […] There is no well established data regarding the gender predilection of POEMS syndrome. […] Data from Japan shows a male predilection of 2.5:1. […] The majority of POEMS syndrome cases have been reported in Japan and China.

• #6 Poems Syndrome, Cryoglobulinemia, and Heavy-Chain Disease | Oncohema Key

  https://oncohemakey.com/poems-syndrome-cryoglobulinemia-and-heavy-chain-disease/

  POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. […] The disease was initially thought to be more common in patients of Japanese descent, given the most numerous initial reports from Japan. However, over the years, large series have also been reported from France, the United States, China, and India. A national survey conducted in Japan in 2003 showed a prevalence of approximately 0.3 per 100,000. […] The diagnosis is established based on a composite of clinical and laboratory features. […] The requirements set forth in Table 101.1 are designed to retain both sensitivity and specificity, potentially erring on the side of specificity. […] The manifestations of extravascular overload occur in 29% to 87% of patients with POEMS syndrome and are not typically associated with severe hypoalbuminemia. […] The course of POEMS syndrome is usually chronic, with modern estimated median survivals of nearly 14 years. […] The number of POEMS features does not affect survival. […] The treatment algorithm is based on the extent of the plasma cell infiltration.

• #7 Ocular Manifestations of Polyneuropathy, Organomegaly, Endocrinopathy, Myeloma Protein, and Skin Changes (POEMS) Syndrome – EyeWiki

  https://eyewiki.org/Ocular_Manifestations_of_Polyneuropathy,_Organomegaly,_Endocrinopathy,_Myeloma_Protein,_and_Skin_Changes_(POEMS)_Syndrome

  POEMS Syndrome, also known as osteosclerotic myeloma, Crow-Fukase syndrome, or Takatsuki syndrome is a paraneoplastic hematologic condition that is characterized by aberrant production of plasma cells. The incidence and prevalence of POEMS syndrome are not as clear as it is a rare condition. In Japan, the alleged country of origin, the prevalence is approximately 0.3 per 100,000 people. Many cases have also been reported in France, China, India, and the USA. A Japanese survey in 2015 indicated a slight male:female ratio of 1.5 with a median age of onset at 54 years of age (range of 21-84). This differs from multiple myeloma, which peaks in the 7th and 8th decades. With an unclear pathogenesis, risk factors for the disease are also largely unknown. […] Studies have shown that optic disc edema, VEGF, and IL-12 levels positively relate to disease activity and may be useful in monitoring the disease. The median time of onset of disease to diagnosis of POEMS syndrome is nearly 13-18 months. The median survival with therapy is approximately 14 years.

• #8 Poems Syndrome, Cryoglobulinemia, and Heavy-Chain Disease | Oncohema Key

  https://oncohemakey.com/poems-syndrome-cryoglobulinemia-and-heavy-chain-disease/

  POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. […] The disease was initially thought to be more common in patients of Japanese descent, given the most numerous initial reports from Japan. However, over the years, large series have also been reported from France, the United States, China, and India. A national survey conducted in Japan in 2003 showed a prevalence of approximately 0.3 per 100,000. […] The diagnosis is established based on a composite of clinical and laboratory features. […] The requirements set forth in Table 101.1 are designed to retain both sensitivity and specificity, potentially erring on the side of specificity. […] The manifestations of extravascular overload occur in 29% to 87% of patients with POEMS syndrome and are not typically associated with severe hypoalbuminemia. […] The course of POEMS syndrome is usually chronic, with modern estimated median survivals of nearly 14 years. […] The number of POEMS features does not affect survival. […] The treatment algorithm is based on the extent of the plasma cell infiltration.

• #9 What Is POEMS Syndrome – Klarity Health Library

  https://my.klarity.health/what-is-poems-syndrome/

  POEMS syndrome is a rare disorder affecting approximately 0.3 in every 100,000 individuals, and it is more commonly seen in males in their 50s and 60s, according to a national survey conducted in 2003 in Japan. […] It was thought to most commonly affect patients of Japanese descent, however, as more research comes to light, it has been reported that populations within countries like France, the USA, China, and India are also significantly affected by POEMS syndrome. […] The prognosis for POEMS syndrome is good in comparison with multiple myeloma. The management and therapies for POEMS syndrome have greatly improved since the 1980s, when the median survival was 33 months. Today, approximately 82% of newly diagnosed patients can expect to live for more than 10 years. […] Scheduling follow-up appointments is necessary to see how well you are doing after your treatment. It’s recommended to follow up at least every 3 months for several years so that doctors can monitor your condition. If there’s a relapse, they can treat and manage your condition as soon as possible.

• #10 POEMS syndrome in Colombia: Clinical findings, therapeutic options and outcomes in a case series | Neurology perspectives

  https://www.elsevier.es/en-revista-neurology-perspectives-17-articulo-poems-syndrome-in-colombia-clinical-S2667049622000515

  POEMS syndrome is a paraneoplastic syndrome associated with a clonal plasma cell neoplasm, in general is a rare disease and its descriptions are based on series and case reports. […] POEMS syndrome has a variable demographic distribution, the largest case series being from Japan, the United States, France, China, and India. In Japan, it has a prevalence of 0.3 cases per 100000 population per year. […] Patients who met the diagnostic criteria for POEMS syndrome proposed by Dispenzieri in 2003 during the period from 2005 to 2021 in the medical consultation of different neurologists specialized in neuromuscular pathology were selected. Also, a review of all published cases of POEMS syndrome in Colombian literature were included. […] This study reviewed the clinical characteristics of 16 patients diagnosed with POEMS syndrome in Colombia. Clinical manifestations were diverse, including motor symptoms, sensory symptoms, constitutional symptoms, adenopathy, signs of water overload, and cutaneous hyperpigmentation.

• #11 POEMS Syndrome: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1097031-overview

  POEMS syndrome is rare, with several hundred cases described in the literature; however, the incidence may be underreported because the syndrome may go unrecognized. […] The onset of POEMS syndrome occurs most frequently in the fifth or sixth decade of life (mean age at onset, 48 y for men and 59 y for women). In 2007 and 2008, however, POEMS syndrome was reported to occur in two 15-year old patients. […] POEMS syndrome is slightly more prevalent among men than women, with a male-to-female ratio of 2.5:1. No specific racial association has been identified, though a preponderance of cases have been reported in the Japanese literature.

• #12 POEMS Syndrome: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1097031-overview

  POEMS syndrome is rare, with several hundred cases described in the literature; however, the incidence may be underreported because the syndrome may go unrecognized. […] The onset of POEMS syndrome occurs most frequently in the fifth or sixth decade of life (mean age at onset, 48 y for men and 59 y for women). In 2007 and 2008, however, POEMS syndrome was reported to occur in two 15-year old patients. […] POEMS syndrome is slightly more prevalent among men than women, with a male-to-female ratio of 2.5:1. No specific racial association has been identified, though a preponderance of cases have been reported in the Japanese literature.

• #13 Orphanet: POEMS syndrome

  https://www.orpha.net/en/disease/detail/2905

  POEMS syndrome is a paraneoplastic syndrome characterized by polyradiculoneuropathy (P), organomegaly (O), endocrinopathy (E), clonal plasma cell disorder (M), and skin changes (S). […] Exact prevalence and incidence rates are not known. The only estimates are from Japan. In this country, prevalence has been reported to be about 1/330,000. Male predilection has been reported (2.5:1). […] The average age of onset is in the 5th or 6th decade. […] The main differential diagnoses are chronic inflammatory demyelinating polyneuropathy, AL amyloidosis and Guillain-Barr syndrome. Monoclonal gammopathy of undetermined significance (MGUS) and should also be considered.

• #14 Ocular Manifestations of Polyneuropathy, Organomegaly, Endocrinopathy, Myeloma Protein, and Skin Changes (POEMS) Syndrome – EyeWiki

  https://eyewiki.org/Ocular_Manifestations_of_Polyneuropathy,_Organomegaly,_Endocrinopathy,_Myeloma_Protein,_and_Skin_Changes_(POEMS)_Syndrome

  POEMS Syndrome, also known as osteosclerotic myeloma, Crow-Fukase syndrome, or Takatsuki syndrome is a paraneoplastic hematologic condition that is characterized by aberrant production of plasma cells. The incidence and prevalence of POEMS syndrome are not as clear as it is a rare condition. In Japan, the alleged country of origin, the prevalence is approximately 0.3 per 100,000 people. Many cases have also been reported in France, China, India, and the USA. A Japanese survey in 2015 indicated a slight male:female ratio of 1.5 with a median age of onset at 54 years of age (range of 21-84). This differs from multiple myeloma, which peaks in the 7th and 8th decades. With an unclear pathogenesis, risk factors for the disease are also largely unknown. […] Studies have shown that optic disc edema, VEGF, and IL-12 levels positively relate to disease activity and may be useful in monitoring the disease. The median time of onset of disease to diagnosis of POEMS syndrome is nearly 13-18 months. The median survival with therapy is approximately 14 years.

• #15 Azthena logo with the word Azthena

  https://www.news-medical.net/health/What-Is-POEMS-Syndrome-An-Overview.aspx

  POEMS syndrome can develop in patients aged 50 or older in extremely rare cases of plasma neoplastic diseases. Notably, men are at a 1.5 times greater risk of POEMS syndrome than women. […] Due to the rarity of POEMS syndrome, it is often difficult to recognize. As a result, the incidence of this paraneoplastic syndrome is likely underrepresented, leading to challenges in its management. […] Both polyneuropathy and monoclonal gammopathy must be present for a POEMS diagnosis. […] Suspected POEMS syndrome cases should undergo a thorough clinical evaluation consisting of a physical exam, medical history, and laboratory testing. […] Increased levels of M-proteins, blood plasma, VEGF, and the aforementioned pro-inflammatory cytokines within the serum may be used to support a POEMS syndrome diagnosis.

• #16

  https://link.springer.com/article/10.1007/s00415-018-9110-6

  POEMS syndrome remains a rare disease and evidence for treatment is largely limited to retrospective cohort studies or case reports. […] POEMS syndrome has a median age of onset in the sixth decade and a slight male preponderance. […] In a study of 113 patients with biopsy confirmed CD treated between 1948 and 2002, 18% met criteria for POEMS syndrome. […] In a study of 87 patients with POEMS syndrome, 10 had CD and 5 had CD-like features on lymph node biopsy. […] Overall survival is dependent on the presence or absence of CD, osteosclerotic lesions, and POEMS syndrome. […] In a Mayo clinic cohort, 5-year survival was very good in patients with unicentric CD (91%) or with MCD with osteosclerotic lesions and POEMS syndrome (90%), good in patients with MCD without POEMS syndrome (65%), and very poor in patients with MCD and POEMS syndrome without osteosclerotic bone lesions (27%).

• #17 POEMS syndrome – Wikipedia

  https://en.wikipedia.org/wiki/POEMS_syndrome

  POEMS syndrome typically begins in middle age the average age at onset is 50 and affects up to twice as many men as women. […] The signs and symptoms of POEMS syndrome are highly variable. This often leads to long delays (e.g. 1318 months) between the onset of initial symptoms and diagnosis. […] POEMS syndrome has been associated with hepatomegaly, splenomegaly, and lymphadenopathy in 5078% of patients. […] 80% of POEMS patients are reported to have extravascular volume overload including, peripheral edema, ascites, pleural effusion, and pericardial effusion. […] Approximately 84% of POEMS syndrome patients have characteristics of several endocrinopathies. […] The diagnosis of POEMS syndrome is based on meeting its two mandatory criteria, meeting at least one of its 3 other major criteria, and meeting at least one of its 6 minor criteria.

• #18 POEMS Syndrome: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1097031-overview

  POEMS syndrome is rare, with several hundred cases described in the literature; however, the incidence may be underreported because the syndrome may go unrecognized. […] The onset of POEMS syndrome occurs most frequently in the fifth or sixth decade of life (mean age at onset, 48 y for men and 59 y for women). In 2007 and 2008, however, POEMS syndrome was reported to occur in two 15-year old patients. […] POEMS syndrome is slightly more prevalent among men than women, with a male-to-female ratio of 2.5:1. No specific racial association has been identified, though a preponderance of cases have been reported in the Japanese literature.

• #19 POEMS Syndrome: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1097031-overview

  POEMS syndrome is rare, with several hundred cases described in the literature; however, the incidence may be underreported because the syndrome may go unrecognized. […] The onset of POEMS syndrome occurs most frequently in the fifth or sixth decade of life (mean age at onset, 48 y for men and 59 y for women). In 2007 and 2008, however, POEMS syndrome was reported to occur in two 15-year old patients. […] POEMS syndrome is slightly more prevalent among men than women, with a male-to-female ratio of 2.5:1. No specific racial association has been identified, though a preponderance of cases have been reported in the Japanese literature.

• #20 Ocular Manifestations of Polyneuropathy, Organomegaly, Endocrinopathy, Myeloma Protein, and Skin Changes (POEMS) Syndrome – EyeWiki

  https://eyewiki.org/Ocular_Manifestations_of_Polyneuropathy,_Organomegaly,_Endocrinopathy,_Myeloma_Protein,_and_Skin_Changes_(POEMS)_Syndrome

  POEMS Syndrome, also known as osteosclerotic myeloma, Crow-Fukase syndrome, or Takatsuki syndrome is a paraneoplastic hematologic condition that is characterized by aberrant production of plasma cells. The incidence and prevalence of POEMS syndrome are not as clear as it is a rare condition. In Japan, the alleged country of origin, the prevalence is approximately 0.3 per 100,000 people. Many cases have also been reported in France, China, India, and the USA. A Japanese survey in 2015 indicated a slight male:female ratio of 1.5 with a median age of onset at 54 years of age (range of 21-84). This differs from multiple myeloma, which peaks in the 7th and 8th decades. With an unclear pathogenesis, risk factors for the disease are also largely unknown. […] Studies have shown that optic disc edema, VEGF, and IL-12 levels positively relate to disease activity and may be useful in monitoring the disease. The median time of onset of disease to diagnosis of POEMS syndrome is nearly 13-18 months. The median survival with therapy is approximately 14 years.

• #21 POEMS Syndrome: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1097031-overview

  POEMS syndrome is rare, with several hundred cases described in the literature; however, the incidence may be underreported because the syndrome may go unrecognized. […] The onset of POEMS syndrome occurs most frequently in the fifth or sixth decade of life (mean age at onset, 48 y for men and 59 y for women). In 2007 and 2008, however, POEMS syndrome was reported to occur in two 15-year old patients. […] POEMS syndrome is slightly more prevalent among men than women, with a male-to-female ratio of 2.5:1. No specific racial association has been identified, though a preponderance of cases have been reported in the Japanese literature.

• #22 POEMS syndrome – Wikipedia

  https://en.wikipedia.org/wiki/POEMS_syndrome

  POEMS syndrome typically begins in middle age the average age at onset is 50 and affects up to twice as many men as women. […] The signs and symptoms of POEMS syndrome are highly variable. This often leads to long delays (e.g. 1318 months) between the onset of initial symptoms and diagnosis. […] POEMS syndrome has been associated with hepatomegaly, splenomegaly, and lymphadenopathy in 5078% of patients. […] 80% of POEMS patients are reported to have extravascular volume overload including, peripheral edema, ascites, pleural effusion, and pericardial effusion. […] Approximately 84% of POEMS syndrome patients have characteristics of several endocrinopathies. […] The diagnosis of POEMS syndrome is based on meeting its two mandatory criteria, meeting at least one of its 3 other major criteria, and meeting at least one of its 6 minor criteria.

• #23 Diagnosis of POEMS syndrome in a patient with long-standing neuropathy | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-diagnosis-poems-syndrome-in-patient-S2173580818301469

  Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare multisystemic disease of paraneoplastic origin, which represents a true clinical challenge. […] Given the rareness, variability, and complexity of forms of onset, diagnosis of POEMS syndrome may be delayed by a median of 13-18 months. […] Therefore, one of the main challenges in the context of this disease is to minimise time to diagnosis. […] Diagnostic delay may entail a decrease in the subsequent response to treatments, increasing sequelae and complications; therefore, one of the main challenges in the context of this disease is to minimise time to diagnosis. […] Clinical suspicion is therefore essential for establishing an early diagnosis. This will provide patients with a specific treatment adapted to their situation, avoiding potential complications and decreasing sequelae, and especially neurological sequelae. Given the greatly variable forms of onset of the syndrome, clinicians should be aware of the multiple manifestations of the disease and perform a directed clinical, analytical, and radiological search.

• #24 Ocular Manifestations of Polyneuropathy, Organomegaly, Endocrinopathy, Myeloma Protein, and Skin Changes (POEMS) Syndrome – EyeWiki

  https://eyewiki.org/Ocular_Manifestations_of_Polyneuropathy,_Organomegaly,_Endocrinopathy,_Myeloma_Protein,_and_Skin_Changes_(POEMS)_Syndrome

  POEMS Syndrome, also known as osteosclerotic myeloma, Crow-Fukase syndrome, or Takatsuki syndrome is a paraneoplastic hematologic condition that is characterized by aberrant production of plasma cells. The incidence and prevalence of POEMS syndrome are not as clear as it is a rare condition. In Japan, the alleged country of origin, the prevalence is approximately 0.3 per 100,000 people. Many cases have also been reported in France, China, India, and the USA. A Japanese survey in 2015 indicated a slight male:female ratio of 1.5 with a median age of onset at 54 years of age (range of 21-84). This differs from multiple myeloma, which peaks in the 7th and 8th decades. With an unclear pathogenesis, risk factors for the disease are also largely unknown. […] Studies have shown that optic disc edema, VEGF, and IL-12 levels positively relate to disease activity and may be useful in monitoring the disease. The median time of onset of disease to diagnosis of POEMS syndrome is nearly 13-18 months. The median survival with therapy is approximately 14 years.

• #25 Diagnosis of POEMS syndrome in a patient with long-standing neuropathy | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-diagnosis-poems-syndrome-in-patient-S2173580818301469

  Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare multisystemic disease of paraneoplastic origin, which represents a true clinical challenge. […] Given the rareness, variability, and complexity of forms of onset, diagnosis of POEMS syndrome may be delayed by a median of 13-18 months. […] Therefore, one of the main challenges in the context of this disease is to minimise time to diagnosis. […] Diagnostic delay may entail a decrease in the subsequent response to treatments, increasing sequelae and complications; therefore, one of the main challenges in the context of this disease is to minimise time to diagnosis. […] Clinical suspicion is therefore essential for establishing an early diagnosis. This will provide patients with a specific treatment adapted to their situation, avoiding potential complications and decreasing sequelae, and especially neurological sequelae. Given the greatly variable forms of onset of the syndrome, clinicians should be aware of the multiple manifestations of the disease and perform a directed clinical, analytical, and radiological search.

• #26 POEMS Syndrome: Clinical Features and Outcomes in a U.K. Cohortlogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/na52146/2020/08/19/poems-syndrome-clinical-features-and-outcomes-uk-cohort

  POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin lesions) is a rare but treatable paraneoplastic disorder of plasma cells associated with sensorimotor polyneuropathy, lambda gammopathy, and elevated cerebrospinal fluid protein and serum VEGF (vascular endothelial growth factor). Missed or delayed diagnosis is common. To investigate characteristics, outcomes, and risk factors, investigators retrospectively assessed data from 100 patients with POEMS syndrome at a U.K. institution who were followed for a median of 59 months. […] Common features included hypogonadotrophic hypogonadism (72%), volume overload (70%), acrocyanosis (46%), lymphadenopathy (42%), weight loss, restrictive lung disease, and pulmonary hypertension. Mean onset-to-diagnosis time was 15 months. Patients diagnosed within 6 months had lower overall neuropathy limitation scores, although more than half of patients were previously misdiagnosed with chronic immune demyelinating polyneuropathy (CIDP).

• #27 POEMS Syndrome: Clinical Features and Outcomes in a U.K. Cohortlogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/na52146/2020/08/19/poems-syndrome-clinical-features-and-outcomes-uk-cohort

  POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin lesions) is a rare but treatable paraneoplastic disorder of plasma cells associated with sensorimotor polyneuropathy, lambda gammopathy, and elevated cerebrospinal fluid protein and serum VEGF (vascular endothelial growth factor). Missed or delayed diagnosis is common. To investigate characteristics, outcomes, and risk factors, investigators retrospectively assessed data from 100 patients with POEMS syndrome at a U.K. institution who were followed for a median of 59 months. […] Common features included hypogonadotrophic hypogonadism (72%), volume overload (70%), acrocyanosis (46%), lymphadenopathy (42%), weight loss, restrictive lung disease, and pulmonary hypertension. Mean onset-to-diagnosis time was 15 months. Patients diagnosed within 6 months had lower overall neuropathy limitation scores, although more than half of patients were previously misdiagnosed with chronic immune demyelinating polyneuropathy (CIDP).

• #28 Frontiers in Medical Case ReportsMedical Research Online Library-Open Access

  https://www.jmedicalcasereports.org/article_html.php?did=13013&issueno=0

  POEMS syndrome is a rare multi-system damaging disease associated with plasma cell disorder?which was coined by Bardwick in 1980 (Bardwick et al., 1980). A national survey conducted in Japan in 2003 indicated that the incidence rate was 0.003% (Nasu et al., 2012). A literature-based study in China shows that, the percentage of misdiagnosis was 85% (Wang et al., 2019), thus its incidence actually may be higher. […] Therefore, early identification of this disease is very important. […] POEMS syndrome is characterized by polyneuropathy, organomegaly, endocrinopathy, M-proteinemia (M-protein), and skin changes as the principal clinical manifestations. […] In 2019, Dispenzieri updated the diagnostic criteria. […] Diagnosis of POEMS syndrome requires 3 of the main criteria to be met, of which Polyneuropathy and Monoclonal plasma cell-proliferative disorder are mandatory, and at least one secondary criterion (Dispenzieri, 2019).

• #29 Treatment and outcomes of POEMS syndrome: changes in the past 20 years | Blood Cancer Journal

  https://www.nature.com/articles/s41408-021-00540-1

  Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare plasma cell dyscrasia with multiorgan involvement. In the past two decades, great progress has been achieved in the diagnosis, treatments, and outcomes of POEMS syndrome. […] A total of 621 patients newly diagnosed with POEMS syndrome between 1 January 2000 and 31 December 2019 at Peking Union Medical College Hospital were included in this study. […] The study cohort was divided into three chronological cohorts that spanned similar time intervals based on the year of diagnosis: 20002009, 20102014, and 20152019, and there were 81, 221, and 319 patients included, respectively, which showed a significantly increasing trend. […] The incidence of hepatomegaly and splenomegaly significantly decreased over time, as well as symptoms related to extravascular volume overload, such as peripheral edema and pleural effusion.

• #30 POEMS Syndrome | Condition | UAMS Health

  https://uamshealth.com/condition/poems-syndrome/

  POEMS syndrome is extremely rare. It affects more men than women, and typically occurs in ones 50s. The exact incidence is unknown. […] A diagnosis of POEMS is determined by the presence of a monoclonal plasma cell disorder, peripheral neuropathy, and one or more of the following: osteosclerotic bone lesions, organomegaly, endocrinopathy, skin changes, increased levels of vascular endothelial growth factor, and swelling. […] Laboratory tests of blood and urine are conducted to check protein and hormone levels. Often vascular endothelial growth factor (VEGF) levels are elevated. […] The effectiveness of treatment is monitored on a regular basis.

• #31 Recent Advances in the Treatment and Supportive Care of POEMS Syndrome

  https://www.mdpi.com/2077-0383/11/23/7011

  POEMS is a rare clonal plasma cell disorder characterized by multi-systemic features that include demyelinating peripheral neuropathy, organomegaly, endocrinopathy, presence of monoclonal proteins (M-protein), and skin changes. […] The diagnosis of POEMS is not always straight forward and often the diagnosis is delayed. It is based on fulfilling mandatory criteria of polyradiculoneuropathy and monoclonal protein and the presence of one major criterion (Castleman disease, sclerotic bone lesions, or elevated VEGF), and at least one minor criterion. […] Due to the rarity of the syndrome, clear guidelines for treatment are still lacking. […] The serum VEGF level is elevated in most patients with POEMS. […] The probability to have POEMS gets higher with greater serum VEFG levels. […] The serum VEGF is markedly elevated and correlates with the activity of the disease.

• #32

  https://link.springer.com/article/10.1007/s10072-023-07064-5

  To investigate the utility of regular serum VEGF (sVEGF) levels assessment in the monitoring of POEMS syndrome. […] Regular sVEGF assessment is a valid biomarker in the prediction of disease reactivation in POEMS syndrome and was particularly useful during the phase of remission. […] The diagnosis requires fulfilment of specific clinical, imaging, and biochemical criteria, including elevated levels of vascular endothelial growth factor (VEGF) detected in more than 85% of cases. […] VEGF measured in either serum or plasma is essential in the diagnostic work-up of POEMS syndrome, since it demonstrates high sensitivity and specificity for the disease. […] Several case series suggest that VEGF correlates with disease activity, but the reported discrepancies between measured levels and relapses, and the raise of VEGF in other illnesses, raise some doubt on the utility of monitoring VEGF during the follow-up of POEMS patients.

• #33 Recent Advances in the Treatment and Supportive Care of POEMS Syndrome

  https://www.mdpi.com/2077-0383/11/23/7011

  Measuring VEGF levels is an essential component of POEMS initial work up and to follow up on response to therapy and disease activity. […] The differential diagnoses to keep in mind are chronic inflammatory polyradiculoneuropathy (CIPD), anti-myelin-associated-glycoprotein (MAG) neuropathy, monoclonal gammopathy of undetermined significance (MGUS), and immunoglobulin light chain (AL) amyloid neuropathy. […] Due to the debilitating nature of POEMS syndrome, early detection and intervention is critical to mitigate the risk of permanent neurologic disability. […] A registry for adults with plasma cells dyscrasia (NCT03717844) is set to be completed in 2029. This study will be also looking into patients with POEMS syndrome, and it will assess their baseline status along with their development over time.

• #34

  https://link.springer.com/article/10.1007/s10072-023-07064-5

  Our study supports the role of sVEGF as a marker of disease activity, treatment response, and prognosis in POEMS syndrome. […] The predictive value of a rise of sVEGF for subsequent relapse had a sensitivity of 58% and a specificity of 100%. […] In patients with refractory disease, the 6-month sVEGF monitoring had little value in the prediction and diagnosis of further clinical worsening, with a sensitivity of 15% and 31%, respectively. […] Even if the main limitation of our study is its retrospective design and the relatively small sample size of patients investigated, it confirms that the 6-month assessment of sVEGF is useful to monitor treatment response and to predict relapses in POEMS syndrome.

• #35

  https://link.springer.com/article/10.1007/s10072-023-07064-5

  Our study supports the role of sVEGF as a marker of disease activity, treatment response, and prognosis in POEMS syndrome. […] The predictive value of a rise of sVEGF for subsequent relapse had a sensitivity of 58% and a specificity of 100%. […] In patients with refractory disease, the 6-month sVEGF monitoring had little value in the prediction and diagnosis of further clinical worsening, with a sensitivity of 15% and 31%, respectively. […] Even if the main limitation of our study is its retrospective design and the relatively small sample size of patients investigated, it confirms that the 6-month assessment of sVEGF is useful to monitor treatment response and to predict relapses in POEMS syndrome.

• #36 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  POEMS syndrome is a rare paraneoplastic syndrome that is caused by an underlying plasma cell disorder. Its main features include polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. […] The diagnosis of POEMS syndrome is made based on a composite of clinical and laboratory features. […] The constellation of neuropathy and any of the following should elicit an in-depth patient evaluation for possible POEMS: a lambda-restricted monoclonal protein, thrombocytosis, anasarca, or papilledema. […] Table 2 illustrates the frequency of POEMS features reported in six of the largest series. The variability between series is most likely a function of retrospective reporting and promptness of diagnosis, rather than ethnic differences. […] Patients with POEMS syndrome are at increased risk for arterial and/or venous thromboses during the course of their disease, with nearly 20% of patients experiencing one of these complications.

• #37 POEMS Syndrome-Clinical Picture and Management

  https://austinpublishinggroup.com/anatomy/fulltext/Anatomy-v9-id1108.php

  One of the most important factors in POEMS mechanism seems to be overproduction of pro inflammatory cytokines eg. Vascular Endothelial Growth Factor (VEGF), tumor necrosis factor-a (TNF- -a) and interleukin -6 (IL-6). […] Although the exact incidence and prevalence is unknown, POEMS is declared to be a rare condition. […] The first and major challenge in making the diagnosis is coming up with an idea that POEMS might be the explanation of patients problems. […] In more than 90% patients creatinine level is within normal limits and significant proteinuria is present in less than 10% cases. […] Based on small studies arterial and venous thrombosis is claimed to appear in 20% of POEMS patients. […] However, thrombotic risk in myeloma seems to be lower than in POEMS. […] The number of clinical features present at diagnosis does not determine survival in POEMS.

• #38 POEMS syndrome: 2021 Update on diagnosis, risk-stratification, and management – PubMed

  https://pubmed.ncbi.nlm.nih.gov/34000085/

  POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. […] Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. […] Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced eGFR. […] POEMS Syndrome / epidemiology

• #39 POEMS Syndrome-Clinical Picture and Management

  https://austinpublishinggroup.com/anatomy/fulltext/Anatomy-v9-id1108.php

  Kourelis et al based on the retrospective analysis of 291 POEMS patients, determined younger age (RR 0.98 [0.96-1.00]), albumin greater than 3.2 g/dL (RR 0.5 [0.32-0.89]) and attainment of complete hematologic response (RR 0.4 [0.2-0.9]) good prognostic factors. […] Extravascular volume overload, pulmonary disorders and ischemic complications are associated with unfavorable prognosis in POEMS. […] Castleman disease presence might negatively influence OS in POEMS patients.

• #40 POEMS Syndrome-Clinical Picture and Management

  https://austinpublishinggroup.com/anatomy/fulltext/Anatomy-v9-id1108.php

  Kourelis et al based on the retrospective analysis of 291 POEMS patients, determined younger age (RR 0.98 [0.96-1.00]), albumin greater than 3.2 g/dL (RR 0.5 [0.32-0.89]) and attainment of complete hematologic response (RR 0.4 [0.2-0.9]) good prognostic factors. […] Extravascular volume overload, pulmonary disorders and ischemic complications are associated with unfavorable prognosis in POEMS. […] Castleman disease presence might negatively influence OS in POEMS patients.

• #41 POEMS Syndrome-Clinical Picture and Management

  https://austinpublishinggroup.com/anatomy/fulltext/Anatomy-v9-id1108.php

  Kourelis et al based on the retrospective analysis of 291 POEMS patients, determined younger age (RR 0.98 [0.96-1.00]), albumin greater than 3.2 g/dL (RR 0.5 [0.32-0.89]) and attainment of complete hematologic response (RR 0.4 [0.2-0.9]) good prognostic factors. […] Extravascular volume overload, pulmonary disorders and ischemic complications are associated with unfavorable prognosis in POEMS. […] Castleman disease presence might negatively influence OS in POEMS patients.

• #42 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  The treatment algorithm is based on the extent of the plasma cell infiltration. […] The course of POEMS syndrome is usually chronic, with a median patient survival time of nearly 14 years. […] Only fingernail clubbing, extravascular volume overload (effusions, edema, and ascites), and respiratory symptoms have been associated with a significantly shorter overall survival time. […] The number of POEMS features does not affect survival. […] In our experience and in a recent report by Li and colleagues, patients with co-existing Castleman disease may have an inferior overall survival compared to patients without it. […] Because no randomized clinical trials have been conducted in patients with POEMS syndrome, treatment recommendations are based on case series and anecdote. […] Despite the relationship between disease response and dropping levels of VEGF, most of the successful outcomes in POEMS have been associated with therapy directed at the underlying clonal plasma cell disorder rather than with antibody treatment that solely targets VEGF.

• #43 SEER Hematopoietic and Lymphoid Neoplasm Database

  https://seer.cancer.gov/seertools/hemelymph/53318e22e4b0626b19275ee2/

  Median overall survival is 14.7 years. […] Most common causes of death are cardiorespiratory failure and infection.

• #44 POEMS Syndrome: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17985-poems-syndrome

  POEMS syndrome is rare. Anywhere from 300 to 300,000 people have it. POEMS syndrome typically affects people in their 50s and 60s. Although it affects people regardless of sex, its more common in men. […] With POEMS, you experience a range of symptoms and effects because of whats called a monoclonal plasma disorder. […] Researchers have discovered that a protein called vascular endothelial growth factor (VEGF) may play a role. The majority of people with POEMS syndrome have high levels of VEGF. Research is ongoing to discover the relationship between VEGF and POEMS syndrome. […] There are no known risk factors for POEMS syndrome. […] POEMS isnt preventable. […] Your outlook depends on factors like which parts of your body are impacted, how early you receive your diagnosis and treatment and how you respond to treatment. […] The median survival rate for most people with POEMS is 14 years. This means that about half of people live less than 14 years and half of people live longer. Your life expectancy depends on your unique disease experience and your treatment.

• #45 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  The treatment algorithm is based on the extent of the plasma cell infiltration. […] The course of POEMS syndrome is usually chronic, with a median patient survival time of nearly 14 years. […] Only fingernail clubbing, extravascular volume overload (effusions, edema, and ascites), and respiratory symptoms have been associated with a significantly shorter overall survival time. […] The number of POEMS features does not affect survival. […] In our experience and in a recent report by Li and colleagues, patients with co-existing Castleman disease may have an inferior overall survival compared to patients without it. […] Because no randomized clinical trials have been conducted in patients with POEMS syndrome, treatment recommendations are based on case series and anecdote. […] Despite the relationship between disease response and dropping levels of VEGF, most of the successful outcomes in POEMS have been associated with therapy directed at the underlying clonal plasma cell disorder rather than with antibody treatment that solely targets VEGF.

• #46 POEMS Syndrome: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17985-poems-syndrome

  POEMS syndrome is rare. Anywhere from 300 to 300,000 people have it. POEMS syndrome typically affects people in their 50s and 60s. Although it affects people regardless of sex, its more common in men. […] With POEMS, you experience a range of symptoms and effects because of whats called a monoclonal plasma disorder. […] Researchers have discovered that a protein called vascular endothelial growth factor (VEGF) may play a role. The majority of people with POEMS syndrome have high levels of VEGF. Research is ongoing to discover the relationship between VEGF and POEMS syndrome. […] There are no known risk factors for POEMS syndrome. […] POEMS isnt preventable. […] Your outlook depends on factors like which parts of your body are impacted, how early you receive your diagnosis and treatment and how you respond to treatment. […] The median survival rate for most people with POEMS is 14 years. This means that about half of people live less than 14 years and half of people live longer. Your life expectancy depends on your unique disease experience and your treatment.

• #47 POEMS syndrome

  https://dermnetnz.org/topics/poems-syndrome

  POEMS syndrome is rare, with only a few hundred cases described in the medical literature. However, it may be underreported due to being under-recognised. Many of these cases are from Japan. […] POEMS syndrome is 2.5 times more common in men than in women. It also presents at a younger age in men, most commonly in their 40s and 50s. […] The diagnosis of POEMS syndrome should be considered in patients with peripheral neuropathy of unknown cause plus a plasma cell disorder. […] POEMS syndrome is a chronic disease. The median survival is around a decade (8-14 years), which is 3 times longer than patients with multiple myeloma. However, many patients are bedridden due to neuropathy (50%).

• #48

  https://link.springer.com/article/10.1007/s00415-018-9110-6

  There is a significant association between the presence of skin changes and abnormal pulmonary function tests, suggesting these patients be meticulously screened for respiratory complications. […] In a study of 291 patients treated at the Mayo clinic between 1974 and 2014, 10-year overall survival was 62%, with younger age, albumin3.2 g/dL and complete haematological response to treatment all associated with better outcomes.

• #49 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  The treatment algorithm is based on the extent of the plasma cell infiltration. […] The course of POEMS syndrome is usually chronic, with a median patient survival time of nearly 14 years. […] Only fingernail clubbing, extravascular volume overload (effusions, edema, and ascites), and respiratory symptoms have been associated with a significantly shorter overall survival time. […] The number of POEMS features does not affect survival. […] In our experience and in a recent report by Li and colleagues, patients with co-existing Castleman disease may have an inferior overall survival compared to patients without it. […] Because no randomized clinical trials have been conducted in patients with POEMS syndrome, treatment recommendations are based on case series and anecdote. […] Despite the relationship between disease response and dropping levels of VEGF, most of the successful outcomes in POEMS have been associated with therapy directed at the underlying clonal plasma cell disorder rather than with antibody treatment that solely targets VEGF.

• #50 POEMS Syndrome-Clinical Picture and Management

  https://austinpublishinggroup.com/anatomy/fulltext/Anatomy-v9-id1108.php

  One of the most important factors in POEMS mechanism seems to be overproduction of pro inflammatory cytokines eg. Vascular Endothelial Growth Factor (VEGF), tumor necrosis factor-a (TNF- -a) and interleukin -6 (IL-6). […] Although the exact incidence and prevalence is unknown, POEMS is declared to be a rare condition. […] The first and major challenge in making the diagnosis is coming up with an idea that POEMS might be the explanation of patients problems. […] In more than 90% patients creatinine level is within normal limits and significant proteinuria is present in less than 10% cases. […] Based on small studies arterial and venous thrombosis is claimed to appear in 20% of POEMS patients. […] However, thrombotic risk in myeloma seems to be lower than in POEMS. […] The number of clinical features present at diagnosis does not determine survival in POEMS.

• #51 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  The treatment algorithm is based on the extent of the plasma cell infiltration. […] The course of POEMS syndrome is usually chronic, with a median patient survival time of nearly 14 years. […] Only fingernail clubbing, extravascular volume overload (effusions, edema, and ascites), and respiratory symptoms have been associated with a significantly shorter overall survival time. […] The number of POEMS features does not affect survival. […] In our experience and in a recent report by Li and colleagues, patients with co-existing Castleman disease may have an inferior overall survival compared to patients without it. […] Because no randomized clinical trials have been conducted in patients with POEMS syndrome, treatment recommendations are based on case series and anecdote. […] Despite the relationship between disease response and dropping levels of VEGF, most of the successful outcomes in POEMS have been associated with therapy directed at the underlying clonal plasma cell disorder rather than with antibody treatment that solely targets VEGF.

• #52 Treatment and outcomes of POEMS syndrome: changes in the past 20 years | Blood Cancer Journal

  https://www.nature.com/articles/s41408-021-00540-1

  The 5-year OS rates in the three periods were 70.0, 85.4, and 92.2%. […] This study provides a thorough view of the changes in the management of POEMS syndrome in the past two decades. It is encouraging to see reduced misdiagnoses and earlier diagnoses. Overall survival increased significantly, mainly due to dramatic advances in non-ASCT therapeutic options. The increasing availability of drugs targeting plasma cells has changed the management of POEMS syndrome, and it is believed that more advances will be made in the future.

• #53 SEER Hematopoietic and Lymphoid Neoplasm Database

  https://seer.cancer.gov/seertools/hemelymph/53318e22e4b0626b19275ee2/

  Median overall survival is 14.7 years. […] Most common causes of death are cardiorespiratory failure and infection.

• #54 Capillary leak phenotype as a major cause of death in patients with POEMS syndrome | Leukemia

  https://www.nature.com/articles/s41375-024-02489-z

  Cause of death (COD) in POEMS (polyneuropathy, organomegaly, endocrinopathies, monoclonal protein and skin changes) syndrome is not well described. […] The underlying pathogenesis of POEMS syndrome remains unclear, however there have been features associated with poorer prognosis. […] While these factors are associated with poorer prognosis, the ultimate cause of death in patients with POEMS syndrome has not been well-described. […] Therefore, the aim of this study was to dissect and describe the causes of death among patients with POEMS syndrome. […] Cause of death was divided into 3 main categories: 1) related to POEMS syndrome and its treatment complications; 2) causes unrelated to POEMS syndrome; and 3) second primary malignancies. […] The distribution of cause of death is shown in Fig. 1. Thirty-two (65%) patients died related to POEMS syndrome or its therapy, and 17 (35%) died from unrelated causes or from second primary malignancy.

• #55 Capillary leak phenotype as a major cause of death in patients with POEMS syndrome | Leukemia

  https://www.nature.com/articles/s41375-024-02489-z

  Of the 32 patients who died related to POEMS syndrome or treatment complications, the most common cause was a capillary leak phenotype (n=19, see below), followed by active treatment complications (n=5), late therapy complications (n=5), and other POEMS related causes (n=3). […] Uncontrolled terminal CLP was observed in 19 patients and was the most common cause of death in our cohort. […] The only notable differences between those patients who died of uncontrolled CLP versus death due to all other known causes were that the CLP group was diagnosed more recently (median diagnosis year 2007 versus 2002, p=0.02), they were younger at time of death (62 versus 65 years), had higher diagnostic platelet counts (469 106/mL versus 415 106/mL, p=0.04), and were more likely to have had ascites at POEMS presentation (53% versus 17%, p=0.01).

• #56 Capillary leak phenotype as a major cause of death in patients with POEMS syndrome | Leukemia

  https://www.nature.com/articles/s41375-024-02489-z

  Of the 32 patients who died related to POEMS syndrome or treatment complications, the most common cause was a capillary leak phenotype (n=19, see below), followed by active treatment complications (n=5), late therapy complications (n=5), and other POEMS related causes (n=3). […] Uncontrolled terminal CLP was observed in 19 patients and was the most common cause of death in our cohort. […] The only notable differences between those patients who died of uncontrolled CLP versus death due to all other known causes were that the CLP group was diagnosed more recently (median diagnosis year 2007 versus 2002, p=0.02), they were younger at time of death (62 versus 65 years), had higher diagnostic platelet counts (469 106/mL versus 415 106/mL, p=0.04), and were more likely to have had ascites at POEMS presentation (53% versus 17%, p=0.01).

• #57 Capillary leak phenotype as a major cause of death in patients with POEMS syndrome | Leukemia

  https://www.nature.com/articles/s41375-024-02489-z

  It is notable that 2 individuals who had prior CLP had been salvaged earlier in their course by corticosteroids, alkylator, bortezomib, and/or ASCT. However, both had recurrence of CLP that did not respond to similar interventions pre-terminally. […] Our study is limited by small sample size and incomplete data. […] Despite these limitations, our findings do bring to the forefront an important entity, the capillary leak phenotype, that is the leading cause of death in patients with POEMS syndrome.

• #58

  https://link.springer.com/article/10.1007/s00415-018-9110-6

  POEMS syndrome remains a rare disease and evidence for treatment is largely limited to retrospective cohort studies or case reports. […] POEMS syndrome has a median age of onset in the sixth decade and a slight male preponderance. […] In a study of 113 patients with biopsy confirmed CD treated between 1948 and 2002, 18% met criteria for POEMS syndrome. […] In a study of 87 patients with POEMS syndrome, 10 had CD and 5 had CD-like features on lymph node biopsy. […] Overall survival is dependent on the presence or absence of CD, osteosclerotic lesions, and POEMS syndrome. […] In a Mayo clinic cohort, 5-year survival was very good in patients with unicentric CD (91%) or with MCD with osteosclerotic lesions and POEMS syndrome (90%), good in patients with MCD without POEMS syndrome (65%), and very poor in patients with MCD and POEMS syndrome without osteosclerotic bone lesions (27%).

• #59 Recent Advances in the Treatment and Supportive Care of POEMS Syndrome

  https://www.mdpi.com/2077-0383/11/23/7011

  Measuring VEGF levels is an essential component of POEMS initial work up and to follow up on response to therapy and disease activity. […] The differential diagnoses to keep in mind are chronic inflammatory polyradiculoneuropathy (CIPD), anti-myelin-associated-glycoprotein (MAG) neuropathy, monoclonal gammopathy of undetermined significance (MGUS), and immunoglobulin light chain (AL) amyloid neuropathy. […] Due to the debilitating nature of POEMS syndrome, early detection and intervention is critical to mitigate the risk of permanent neurologic disability. […] A registry for adults with plasma cells dyscrasia (NCT03717844) is set to be completed in 2029. This study will be also looking into patients with POEMS syndrome, and it will assess their baseline status along with their development over time.

• #60 POEMS syndrome : University College London Hospitals NHS Foundation Trust

  https://www.uclh.nhs.uk/our-services/find-service/cancer-services/blood-cancers-clinical-haematology/blood-diseases-types-and-services/blood-cancers/poems-syndrome

  POEMS syndrome is a rare condition caused by the body producing abnormal plasma cells (a type of blood cell which produces antibodies to fight off infections). This blood disorder affects multiple organs in the body. […] UCLH currently manages one of the largest reported group of POEMS patients in Europe, with approximately 150 people attending the clinic. […] We conduct research into POEMS syndrome to understand more about UK POEMS patients, mechanisms of disease and how we treat the condition most effectively.

• #61 POEMS syndrome – Doctors and departments – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/poems-syndrome/doctors-departments/ddc-20352680

  Mayo Clinic doctors are actively involved in research on POEMS syndrome risk factors and outcomes. […] See a list of publications about POEMS syndrome by Mayo Clinic doctors on PubMed, a service of the National Library of Medicine.

• #62 Treatment and outcomes of POEMS syndrome: changes in the past 20 years | Blood Cancer Journal

  https://www.nature.com/articles/s41408-021-00540-1

  The 5-year OS rates in the three periods were 70.0, 85.4, and 92.2%. […] This study provides a thorough view of the changes in the management of POEMS syndrome in the past two decades. It is encouraging to see reduced misdiagnoses and earlier diagnoses. Overall survival increased significantly, mainly due to dramatic advances in non-ASCT therapeutic options. The increasing availability of drugs targeting plasma cells has changed the management of POEMS syndrome, and it is believed that more advances will be made in the future.

• #63 POEMS syndrome epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/POEMS_syndrome_epidemiology_and_demographics

  The precise incidence of POEMS syndrome is difficult to determine due to the complexity of the syndrome presentation and we have no well established data regarding the incidence. […] Prevalence of POEMS syndrome in Japan is reported to be about 0.3 per 100,000 individuals. […] The median age at presentation is 51 years. […] There is no racial predilection to POEMS syndrome. […] There is no well established data regarding the gender predilection of POEMS syndrome. […] Data from Japan shows a male predilection of 2.5:1. […] The majority of POEMS syndrome cases have been reported in Japan and China.

• #64 Diagnosis of POEMS syndrome in a patient with long-standing neuropathy | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-diagnosis-poems-syndrome-in-patient-S2173580818301469

  Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare multisystemic disease of paraneoplastic origin, which represents a true clinical challenge. […] Given the rareness, variability, and complexity of forms of onset, diagnosis of POEMS syndrome may be delayed by a median of 13-18 months. […] Therefore, one of the main challenges in the context of this disease is to minimise time to diagnosis. […] Diagnostic delay may entail a decrease in the subsequent response to treatments, increasing sequelae and complications; therefore, one of the main challenges in the context of this disease is to minimise time to diagnosis. […] Clinical suspicion is therefore essential for establishing an early diagnosis. This will provide patients with a specific treatment adapted to their situation, avoiding potential complications and decreasing sequelae, and especially neurological sequelae. Given the greatly variable forms of onset of the syndrome, clinicians should be aware of the multiple manifestations of the disease and perform a directed clinical, analytical, and radiological search.

• #65 Diagnosis of POEMS syndrome in a patient with long-standing neuropathy | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-diagnosis-poems-syndrome-in-patient-S2173580818301469

  Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare multisystemic disease of paraneoplastic origin, which represents a true clinical challenge. […] Given the rareness, variability, and complexity of forms of onset, diagnosis of POEMS syndrome may be delayed by a median of 13-18 months. […] Therefore, one of the main challenges in the context of this disease is to minimise time to diagnosis. […] Diagnostic delay may entail a decrease in the subsequent response to treatments, increasing sequelae and complications; therefore, one of the main challenges in the context of this disease is to minimise time to diagnosis. […] Clinical suspicion is therefore essential for establishing an early diagnosis. This will provide patients with a specific treatment adapted to their situation, avoiding potential complications and decreasing sequelae, and especially neurological sequelae. Given the greatly variable forms of onset of the syndrome, clinicians should be aware of the multiple manifestations of the disease and perform a directed clinical, analytical, and radiological search.

• #66 Diagnosis of POEMS syndrome in a patient with long-standing neuropathy | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-diagnosis-poems-syndrome-in-patient-S2173580818301469

  Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare multisystemic disease of paraneoplastic origin, which represents a true clinical challenge. […] Given the rareness, variability, and complexity of forms of onset, diagnosis of POEMS syndrome may be delayed by a median of 13-18 months. […] Therefore, one of the main challenges in the context of this disease is to minimise time to diagnosis. […] Diagnostic delay may entail a decrease in the subsequent response to treatments, increasing sequelae and complications; therefore, one of the main challenges in the context of this disease is to minimise time to diagnosis. […] Clinical suspicion is therefore essential for establishing an early diagnosis. This will provide patients with a specific treatment adapted to their situation, avoiding potential complications and decreasing sequelae, and especially neurological sequelae. Given the greatly variable forms of onset of the syndrome, clinicians should be aware of the multiple manifestations of the disease and perform a directed clinical, analytical, and radiological search.

• #67 Recent Advances in the Treatment and Supportive Care of POEMS Syndrome

  https://www.mdpi.com/2077-0383/11/23/7011

  POEMS is a rare clonal plasma cell disorder characterized by multi-systemic features that include demyelinating peripheral neuropathy, organomegaly, endocrinopathy, presence of monoclonal proteins (M-protein), and skin changes. […] The diagnosis of POEMS is not always straight forward and often the diagnosis is delayed. It is based on fulfilling mandatory criteria of polyradiculoneuropathy and monoclonal protein and the presence of one major criterion (Castleman disease, sclerotic bone lesions, or elevated VEGF), and at least one minor criterion. […] Due to the rarity of the syndrome, clear guidelines for treatment are still lacking. […] The serum VEGF level is elevated in most patients with POEMS. […] The probability to have POEMS gets higher with greater serum VEFG levels. […] The serum VEGF is markedly elevated and correlates with the activity of the disease.

• #68

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  We regard POEMS syndrome as a hematological/neurological emergency and complete diagnostic testing as soon as possible to enable rapid institution of therapy. […] A parallel multidisciplinary approach is advantageous and efficient. […] Future clinical trials are welcomed in this setting where evidence is limited.

• #69

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  We regard POEMS syndrome as a hematological/neurological emergency and complete diagnostic testing as soon as possible to enable rapid institution of therapy. […] A parallel multidisciplinary approach is advantageous and efficient. […] Future clinical trials are welcomed in this setting where evidence is limited.

• #70 Recent Advances in the Treatment and Supportive Care of POEMS Syndrome

  https://www.mdpi.com/2077-0383/11/23/7011

  Measuring VEGF levels is an essential component of POEMS initial work up and to follow up on response to therapy and disease activity. […] The differential diagnoses to keep in mind are chronic inflammatory polyradiculoneuropathy (CIPD), anti-myelin-associated-glycoprotein (MAG) neuropathy, monoclonal gammopathy of undetermined significance (MGUS), and immunoglobulin light chain (AL) amyloid neuropathy. […] Due to the debilitating nature of POEMS syndrome, early detection and intervention is critical to mitigate the risk of permanent neurologic disability. […] A registry for adults with plasma cells dyscrasia (NCT03717844) is set to be completed in 2029. This study will be also looking into patients with POEMS syndrome, and it will assess their baseline status along with their development over time.

• #71 POEMS syndrome – Autoimmune Association

  https://autoimmune.org/disease-information/poems-syndrome/

  POEMS syndrome is a rare autoimmune related blood disorder that damages your nerves and affects many other parts of the body. […] POEMS syndrome can be misdiagnosed because the signs and symptoms mimic those of other disorders. […] POEMS syndrome progresses rapidly and may become life-threatening, so early diagnosis is important. […] POEMS syndrome is a rare, mulitisystem disorder. […] The underlying cause of the disorder is not well understood. […] POEMS syndrome is a chronic disorder, with a median survival time of 8-14 years. […] There is no standard treatment; management depends on the underlying plasma cell disorder and may include radiation therapy, chemotherapy, and/or hematopoietic cell transplantation. […] The cause of POEMS syndrome is not well understood. […] POEMS syndrome is a paraneoplastic disorder, which means that the signs and symptoms of the syndrome appear as a manifestation of the plasma cell disorder that the patient has. […] It is associated with a chronic overproduction of some substances known as pro-inflammatory cytokines.

• #72 Recent Advances in the Treatment and Supportive Care of POEMS Syndrome

  https://www.mdpi.com/2077-0383/11/23/7011

  Measuring VEGF levels is an essential component of POEMS initial work up and to follow up on response to therapy and disease activity. […] The differential diagnoses to keep in mind are chronic inflammatory polyradiculoneuropathy (CIPD), anti-myelin-associated-glycoprotein (MAG) neuropathy, monoclonal gammopathy of undetermined significance (MGUS), and immunoglobulin light chain (AL) amyloid neuropathy. […] Due to the debilitating nature of POEMS syndrome, early detection and intervention is critical to mitigate the risk of permanent neurologic disability. […] A registry for adults with plasma cells dyscrasia (NCT03717844) is set to be completed in 2029. This study will be also looking into patients with POEMS syndrome, and it will assess their baseline status along with their development over time.

• #73 POEMS syndrome – Autoimmune Association

  https://autoimmune.org/disease-information/poems-syndrome/

  POEMS syndrome is a rare autoimmune related blood disorder that damages your nerves and affects many other parts of the body. […] POEMS syndrome can be misdiagnosed because the signs and symptoms mimic those of other disorders. […] POEMS syndrome progresses rapidly and may become life-threatening, so early diagnosis is important. […] POEMS syndrome is a rare, mulitisystem disorder. […] The underlying cause of the disorder is not well understood. […] POEMS syndrome is a chronic disorder, with a median survival time of 8-14 years. […] There is no standard treatment; management depends on the underlying plasma cell disorder and may include radiation therapy, chemotherapy, and/or hematopoietic cell transplantation. […] The cause of POEMS syndrome is not well understood. […] POEMS syndrome is a paraneoplastic disorder, which means that the signs and symptoms of the syndrome appear as a manifestation of the plasma cell disorder that the patient has. […] It is associated with a chronic overproduction of some substances known as pro-inflammatory cytokines.

• #74 POEMS syndrome

  https://dermnetnz.org/topics/poems-syndrome

  POEMS syndrome is rare, with only a few hundred cases described in the medical literature. However, it may be underreported due to being under-recognised. Many of these cases are from Japan. […] POEMS syndrome is 2.5 times more common in men than in women. It also presents at a younger age in men, most commonly in their 40s and 50s. […] The diagnosis of POEMS syndrome should be considered in patients with peripheral neuropathy of unknown cause plus a plasma cell disorder. […] POEMS syndrome is a chronic disease. The median survival is around a decade (8-14 years), which is 3 times longer than patients with multiple myeloma. However, many patients are bedridden due to neuropathy (50%).

• #75 POEMS syndrome – Wikipedia

  https://en.wikipedia.org/wiki/POEMS_syndrome

  POEMS syndrome typically begins in middle age the average age at onset is 50 and affects up to twice as many men as women. […] The signs and symptoms of POEMS syndrome are highly variable. This often leads to long delays (e.g. 1318 months) between the onset of initial symptoms and diagnosis. […] POEMS syndrome has been associated with hepatomegaly, splenomegaly, and lymphadenopathy in 5078% of patients. […] 80% of POEMS patients are reported to have extravascular volume overload including, peripheral edema, ascites, pleural effusion, and pericardial effusion. […] Approximately 84% of POEMS syndrome patients have characteristics of several endocrinopathies. […] The diagnosis of POEMS syndrome is based on meeting its two mandatory criteria, meeting at least one of its 3 other major criteria, and meeting at least one of its 6 minor criteria.

• #76

  https://journals.lww.com/md-journal/fulltext/2024/09060/a_case_of_paraprotein_negative_poems_syndrome_.102.aspx

  POEMS syndrome is a rare monoclonal plasma cell disease. The diagnosis of POEMS requires polyradiculoneuropathy and monoclonal plasma proliferating as 2 mandatory criteria, at least 1 of the major criteria (Castleman disease, elevated vascular endothelial growth factor level, and sclerotic bone lesion), and at least 1 of the minor criteria (organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilledema, and thrombocytosis/polycythemia). […] POEMS syndrome is easy to misdiagnose and delays the diagnosis, especially M protein-negative POEMS syndrome due to the absence of clonal plasma cells. […] The pathogenesis of POEMS syndrome is not completely clear, but it has been hypothesized that abnormally high serum concentrations of VEGF trigger a variety of symptoms. […] Compared to classical POEMS syndrome, M protein-negative POEMS syndrome has rarely been reported, and its incidence is unknown. Given the scarcity of the disease, it is more prone to misdiagnosis and delayed diagnosis than the M protein-positive POEMS syndrome.

• #77 POEMS Syndrome-Clinical Picture and Management

  https://austinpublishinggroup.com/anatomy/fulltext/Anatomy-v9-id1108.php

  One of the most important factors in POEMS mechanism seems to be overproduction of pro inflammatory cytokines eg. Vascular Endothelial Growth Factor (VEGF), tumor necrosis factor-a (TNF- -a) and interleukin -6 (IL-6). […] Although the exact incidence and prevalence is unknown, POEMS is declared to be a rare condition. […] The first and major challenge in making the diagnosis is coming up with an idea that POEMS might be the explanation of patients problems. […] In more than 90% patients creatinine level is within normal limits and significant proteinuria is present in less than 10% cases. […] Based on small studies arterial and venous thrombosis is claimed to appear in 20% of POEMS patients. […] However, thrombotic risk in myeloma seems to be lower than in POEMS. […] The number of clinical features present at diagnosis does not determine survival in POEMS.

• #78 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  POEMS syndrome is a rare paraneoplastic syndrome that is caused by an underlying plasma cell disorder. Its main features include polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. […] The diagnosis of POEMS syndrome is made based on a composite of clinical and laboratory features. […] The constellation of neuropathy and any of the following should elicit an in-depth patient evaluation for possible POEMS: a lambda-restricted monoclonal protein, thrombocytosis, anasarca, or papilledema. […] Table 2 illustrates the frequency of POEMS features reported in six of the largest series. The variability between series is most likely a function of retrospective reporting and promptness of diagnosis, rather than ethnic differences. […] Patients with POEMS syndrome are at increased risk for arterial and/or venous thromboses during the course of their disease, with nearly 20% of patients experiencing one of these complications.

• #79

  https://journals.lww.com/hemasphere/fulltext/2022/11000/comprehensive_diagnosis_and_management_of_poems.12.aspx

  We regard POEMS syndrome as a hematological/neurological emergency and complete diagnostic testing as soon as possible to enable rapid institution of therapy. […] A parallel multidisciplinary approach is advantageous and efficient. […] Future clinical trials are welcomed in this setting where evidence is limited.

• #80 POEMS syndrome: 2021 Update on diagnosis, risk-stratification, and management – PubMed

  https://pubmed.ncbi.nlm.nih.gov/34000085/

  POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. […] Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. […] Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced eGFR. […] POEMS Syndrome / epidemiology

• #81 POEMS syndrome – Autoimmune Association

  https://autoimmune.org/disease-information/poems-syndrome/

  POEMS syndrome is a rare autoimmune related blood disorder that damages your nerves and affects many other parts of the body. […] POEMS syndrome can be misdiagnosed because the signs and symptoms mimic those of other disorders. […] POEMS syndrome progresses rapidly and may become life-threatening, so early diagnosis is important. […] POEMS syndrome is a rare, mulitisystem disorder. […] The underlying cause of the disorder is not well understood. […] POEMS syndrome is a chronic disorder, with a median survival time of 8-14 years. […] There is no standard treatment; management depends on the underlying plasma cell disorder and may include radiation therapy, chemotherapy, and/or hematopoietic cell transplantation. […] The cause of POEMS syndrome is not well understood. […] POEMS syndrome is a paraneoplastic disorder, which means that the signs and symptoms of the syndrome appear as a manifestation of the plasma cell disorder that the patient has. […] It is associated with a chronic overproduction of some substances known as pro-inflammatory cytokines.

• #82 POEMS syndrome: 2021 Update on diagnosis, risk-stratification, and management – PubMed

  https://pubmed.ncbi.nlm.nih.gov/34000085/

  POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. […] Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. […] Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced eGFR. […] POEMS Syndrome / epidemiology

• #83 The unclear role of VEGF in POEMS syndrome: therapeutic implications of neoangiogenesis in a rare plasma cell disorder

  https://www.oaepublish.com/articles/2394-4722.2021.106

  POEMS syndrome is a rare paraneoplastic disorder due to an underlying clone of aberrant plasma cells. […] The epidemiology of the disease is not well established given its very low incidence. […] Even if the role of VEGF in the pathogenesis of POEMS remains unclear, it is still the most important cytokine correlating with disease activity. […] The evidence of high levels of VEGF in serum and plasma of patients with POEMS guided the idea that the syndrome could be successfully treated with drugs directed against this cytokine. […] POEMS syndrome is a rare paraneoplastic disease with a multiorgan involvement which could result fatal in some cases. […] Due to the rarity of the disease, the main information about its treatment in general is based more on real-life experience than on randomized trials. […] Meanwhile, further efforts are required to better identify the real role of VEGF and clarify whether it can be used in clinical practice.

• #84 The unclear role of VEGF in POEMS syndrome: therapeutic implications of neoangiogenesis in a rare plasma cell disorder

  https://www.oaepublish.com/articles/2394-4722.2021.106

  POEMS syndrome is a rare paraneoplastic disorder due to an underlying clone of aberrant plasma cells. […] The epidemiology of the disease is not well established given its very low incidence. […] Even if the role of VEGF in the pathogenesis of POEMS remains unclear, it is still the most important cytokine correlating with disease activity. […] The evidence of high levels of VEGF in serum and plasma of patients with POEMS guided the idea that the syndrome could be successfully treated with drugs directed against this cytokine. […] POEMS syndrome is a rare paraneoplastic disease with a multiorgan involvement which could result fatal in some cases. […] Due to the rarity of the disease, the main information about its treatment in general is based more on real-life experience than on randomized trials. […] Meanwhile, further efforts are required to better identify the real role of VEGF and clarify whether it can be used in clinical practice.

• #85 The unclear role of VEGF in POEMS syndrome: therapeutic implications of neoangiogenesis in a rare plasma cell disorder

  https://www.oaepublish.com/articles/2394-4722.2021.106

  POEMS syndrome is a rare paraneoplastic disorder due to an underlying clone of aberrant plasma cells. […] The epidemiology of the disease is not well established given its very low incidence. […] Even if the role of VEGF in the pathogenesis of POEMS remains unclear, it is still the most important cytokine correlating with disease activity. […] The evidence of high levels of VEGF in serum and plasma of patients with POEMS guided the idea that the syndrome could be successfully treated with drugs directed against this cytokine. […] POEMS syndrome is a rare paraneoplastic disease with a multiorgan involvement which could result fatal in some cases. […] Due to the rarity of the disease, the main information about its treatment in general is based more on real-life experience than on randomized trials. […] Meanwhile, further efforts are required to better identify the real role of VEGF and clarify whether it can be used in clinical practice.

• #86

  https://insight.jci.org/articles/view/151482

  POEMS syndrome is a rare monoclonal plasma cell disorder, with unique symptoms distinct from those of other plasma cell neoplasms, including high serum VEGF levels. […] Because the prospective isolation of POEMS clones has not yet been successful, their real nature remains unclear. […] In contrast to the clinical advances in diagnostic and therapeutic strategies, limited information is currently available on the pathogenesis of POEMS syndrome. […] Plasma cells in POEMS syndrome account for approximately 2%5% of total BM nucleated cells, which is markedly lower than that in multiple myeloma (MM). […] Our recent genome sequencing of BM plasma cells in POEMS syndrome identified 7 recurrently mutated genes: KLHL6, LTB, EHD1, EML4, HEPHL1, HIPK1, and PCDH10. […] Importantly, none of the driver gene mutations frequently found in MM were identified in either of these studies, suggesting that the mutational characteristics of POEMS syndrome are different from those of other plasma cell neoplasms.

• #87

  https://insight.jci.org/articles/view/151482

  POEMS syndrome is a rare monoclonal plasma cell disorder, with unique symptoms distinct from those of other plasma cell neoplasms, including high serum VEGF levels. […] Because the prospective isolation of POEMS clones has not yet been successful, their real nature remains unclear. […] In contrast to the clinical advances in diagnostic and therapeutic strategies, limited information is currently available on the pathogenesis of POEMS syndrome. […] Plasma cells in POEMS syndrome account for approximately 2%5% of total BM nucleated cells, which is markedly lower than that in multiple myeloma (MM). […] Our recent genome sequencing of BM plasma cells in POEMS syndrome identified 7 recurrently mutated genes: KLHL6, LTB, EHD1, EML4, HEPHL1, HIPK1, and PCDH10. […] Importantly, none of the driver gene mutations frequently found in MM were identified in either of these studies, suggesting that the mutational characteristics of POEMS syndrome are different from those of other plasma cell neoplasms.

• #88

  https://insight.jci.org/articles/view/151482

  However, monoclonal plasma cells from patients with POEMS syndrome (referred to here in as POEMS clones) have not yet been identified because of their small size, and thus, their characteristics remain obscure. […] The proportion of POEMS clones among total BM plasma cells was much smaller than that of MM and MGUS clones, as we had expected. […] Our single-cell study confirmed that POEMS clones frequently have amino acid changes in the CDR1 and FR2 regions of IGLV1-40 and IGLV1-44. […] VEGF was not elevated in both clonal and nonclonal plasma cells in POEMS syndrome, which provides the first direct evidence to our knowledge that VEGF is not produced by plasma cells in POEMS syndrome, and, therefore, the source of VEGF remains unknown. […] Identification of the POEMS clonespecific surface marker phenotype allows us to directly access POEMS clones for further characterization of this poorly understood plasma cell disorder and monitor the therapeutic responses.

• #89

  https://insight.jci.org/articles/view/151482

  However, monoclonal plasma cells from patients with POEMS syndrome (referred to here in as POEMS clones) have not yet been identified because of their small size, and thus, their characteristics remain obscure. […] The proportion of POEMS clones among total BM plasma cells was much smaller than that of MM and MGUS clones, as we had expected. […] Our single-cell study confirmed that POEMS clones frequently have amino acid changes in the CDR1 and FR2 regions of IGLV1-40 and IGLV1-44. […] VEGF was not elevated in both clonal and nonclonal plasma cells in POEMS syndrome, which provides the first direct evidence to our knowledge that VEGF is not produced by plasma cells in POEMS syndrome, and, therefore, the source of VEGF remains unknown. […] Identification of the POEMS clonespecific surface marker phenotype allows us to directly access POEMS clones for further characterization of this poorly understood plasma cell disorder and monitor the therapeutic responses.

• #90

  https://insight.jci.org/articles/view/151482

  However, monoclonal plasma cells from patients with POEMS syndrome (referred to here in as POEMS clones) have not yet been identified because of their small size, and thus, their characteristics remain obscure. […] The proportion of POEMS clones among total BM plasma cells was much smaller than that of MM and MGUS clones, as we had expected. […] Our single-cell study confirmed that POEMS clones frequently have amino acid changes in the CDR1 and FR2 regions of IGLV1-40 and IGLV1-44. […] VEGF was not elevated in both clonal and nonclonal plasma cells in POEMS syndrome, which provides the first direct evidence to our knowledge that VEGF is not produced by plasma cells in POEMS syndrome, and, therefore, the source of VEGF remains unknown. […] Identification of the POEMS clonespecific surface marker phenotype allows us to directly access POEMS clones for further characterization of this poorly understood plasma cell disorder and monitor the therapeutic responses.

• #91 The unclear role of VEGF in POEMS syndrome: therapeutic implications of neoangiogenesis in a rare plasma cell disorder

  https://www.oaepublish.com/articles/2394-4722.2021.106

  POEMS syndrome is a rare paraneoplastic disorder due to an underlying clone of aberrant plasma cells. […] The epidemiology of the disease is not well established given its very low incidence. […] Even if the role of VEGF in the pathogenesis of POEMS remains unclear, it is still the most important cytokine correlating with disease activity. […] The evidence of high levels of VEGF in serum and plasma of patients with POEMS guided the idea that the syndrome could be successfully treated with drugs directed against this cytokine. […] POEMS syndrome is a rare paraneoplastic disease with a multiorgan involvement which could result fatal in some cases. […] Due to the rarity of the disease, the main information about its treatment in general is based more on real-life experience than on randomized trials. […] Meanwhile, further efforts are required to better identify the real role of VEGF and clarify whether it can be used in clinical practice.

• #92 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  The treatment algorithm is based on the extent of the plasma cell infiltration. […] The course of POEMS syndrome is usually chronic, with a median patient survival time of nearly 14 years. […] Only fingernail clubbing, extravascular volume overload (effusions, edema, and ascites), and respiratory symptoms have been associated with a significantly shorter overall survival time. […] The number of POEMS features does not affect survival. […] In our experience and in a recent report by Li and colleagues, patients with co-existing Castleman disease may have an inferior overall survival compared to patients without it. […] Because no randomized clinical trials have been conducted in patients with POEMS syndrome, treatment recommendations are based on case series and anecdote. […] Despite the relationship between disease response and dropping levels of VEGF, most of the successful outcomes in POEMS have been associated with therapy directed at the underlying clonal plasma cell disorder rather than with antibody treatment that solely targets VEGF.

• #93 A Review of POEMS Syndrome

  https://www.cancernetwork.com/view/review-poems-syndrome

  The treatment algorithm is based on the extent of the plasma cell infiltration. […] The course of POEMS syndrome is usually chronic, with a median patient survival time of nearly 14 years. […] Only fingernail clubbing, extravascular volume overload (effusions, edema, and ascites), and respiratory symptoms have been associated with a significantly shorter overall survival time. […] The number of POEMS features does not affect survival. […] In our experience and in a recent report by Li and colleagues, patients with co-existing Castleman disease may have an inferior overall survival compared to patients without it. […] Because no randomized clinical trials have been conducted in patients with POEMS syndrome, treatment recommendations are based on case series and anecdote. […] Despite the relationship between disease response and dropping levels of VEGF, most of the successful outcomes in POEMS have been associated with therapy directed at the underlying clonal plasma cell disorder rather than with antibody treatment that solely targets VEGF.

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