Materiały źródłowe

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

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

  There are no known risk factors for POEMS syndrome. […] POEMS isn’t preventable.

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

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

  We report a successful diagnosis of POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes) patient who presented with fever, diarrhea and progressive fatigue in the lower limbs. […] Therefore, early identification of this disease is very important. […] The aim of this case report is to enhance the awareness of POEMS syndrome. […] Therefore, when clinicians encounter a patient with unexplained peripheral neuropathy or multisystem disorders, and also suffers from diabetes or other endocrinopathy, detailed examinations should be performed to rule out POEMS syndrome. […] In clinical practice, the study and appreciation of POEMS syndrome require strengthening, so as to achieve early diagnosis and treatment of the disease, and thereby improve prognosis.

• #1 Oncology Letters

  https://www.spandidos-publications.com/10.3892/ol.2017.6904

  POEMS syndrome is potentially fatal and is associated with a substantial deterioration in quality of life through neuropathy, anasarca, thromboembolic events and cachexia. […] Early diagnosis and a multidisciplinary approach may increase the likelihood of reducing long-term irreversible morbidity. […] The present study recommends that more case reports concerning abnormal symptom-associated POEMS syndrome should be considered to improve the future diagnosis and treatment of POEMS syndrome, and enhance understanding of the syndrome.

• #1 POEMS syndrome survivor: It’s OK to ask for help | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/multiple-myeloma-poems-syndrome-survivor–its-ok-to-ask-for-help.h00-159223356.html

  Most doctors have never even heard of POEMS syndrome, much less treated it. And some neurologists wouldn’t recognize the disease if they saw it. It’s often misdiagnosed as CIDP, so patients with moderate to severe polyneuropathy who are being treated for CIDP should be tested for POEMS. Especially if the CIDP treatments aren’t helping. […] I also wish I hadn’t waited until my last surgery in September 2015 to reach out to the Psychiatric Oncology department at MD Anderson. I should’ve done that right from the beginning. Cancer tears you apart emotionally and physically. It can tear your family apart, too. So, start therapy right away, and if you have children, have them see a therapist as well. […] There is so much support out there, from people who really want to help and who know how to. So, turn to them. Just ask. You won’t regret it.

• #1 POEMS syndrome | ABC Medical Center

  https://centromedicoabc.com/en/padecimientos/poems-syndrome/

  POEMS syndrome, being a chronic condition, cannot be cured, so therapy focuses on controlling the symptoms, delaying the progression of the disease, and preventing serious complications through: […] At ABC Medical Centers Internal Medicine Department, we offer health care services with the highest quality and safety, from the prevention, diagnosis, timely treatment, and monitoring of infectious, respiratory, endocrinological, dermatological, rheumatic, nephrological, gastrointestinal, and hematological pathologies of both chronic-degenerative diseases and acute conditions, through a comprehensive and multidisciplinary model.

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

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9741379/

  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. […] Due to the rarity of the syndrome, clear guidelines for treatment are still lacking. Immediate treatment targeting the underlying plasma cell proliferation results in a dramatic response in most patients. The key is early diagnosis and immediate anti-plasma cell directed therapy for the best clinical outcomes. […] For patients with disseminated disease as defined by bone marrow involvement or more than three osteosclerotic bone lesions, high-dose chemotherapy with autologous hematopoietic stem cell transplant (ASCT) yields durable responses and is the preferred treatment in eligible patients.

• #1 POEMS Syndrome Patient Goes from Immobility to Recovery | Dana-Farber Cancer Institute

  https://blog.dana-farber.org/insight/2017/10/poems-syndrome-patient-goes-immobility-recovery/

  Aby zapobiec nawrotowi, najlepszym sposobem jest przeszczep komórek macierzystych z użyciem własnych komórek, relacjonuje Anyanwu. […] Większość pacjentów z zespołem POEMS reaguje na leczenie, jednak stopień ich powrotu do zdrowia różni się znacznie, zauważa Paba-Prada. […] Niektórzy wracają do pełni zdrowia, inni nie. Niektórzy mają pozostałości neuropatii. Neuropatia Anyanwua nadal stopniowo ustępuje.

• #1

  https://haematologica.org/article/view/haematol.2023.283719

  The results were in fact impressive in terms of PFS, time to retreatment and OS for patients affected by POEMS syndrome who underwent transplantation, confirming that aPBSCT is an effective and safe procedure in patients with this syndrome, independently of the mobilizing treatment. […] Patients unsuitable to undergo front-line aPBSCT because of comorbidity could be rescued with cyclophosphamide as a therapeutic and mobilizing agent.

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

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9741379/

  Overall, ASCT is a safe and effective choice in many patients and can provide durable response in about 90% of eligible patients. […] The recommended treatment is lenalidomide (15-25 mg orally) taken days 1-21 every 28 days with weekly dexamethasone 20-40 mg. […] The mainstay of treatment is anti-plasma cell therapy such as IMiD or ASCT which will relieve polyneuropathy in most if not all of patients over times. […] The morbidity and mortality can be reduced with an early diagnosis and prompt treatment. Indications for poor outcomes are fingernail clubbing, respiratory symptoms, and extravascular fluid overload (ascites, pleural effusion, peripheral edema) and lack of complete hematologic response after ASCT.

• #1 POEMS Syndrome Treatment & Management: Medical Care, Surgical Care, Consultations

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

  In a study from China, 31 patients received 12 cycles of oral melphalan (10 mg/m2 body surface area [BSA]) plus oral dexamethasone (40 mg/d) on days 1-4 of every 28 days. Prophylaxis with a proton-pump inhibitor was added on days 1 to 5. Doses were adjusted according to side effects and renal function. Good hematologic and neurologic responses were reported. […] For patients with POEMS and pulmonary hypertension, high-dose steroid treatment appears to provide improvement, as confirmed by sequential hemodynamic studies. […] Systemic corticosteroids as first-line treatment in pulmonary hypertension associated with POEMS syndrome.

• #1 What is the best first-line treatment for POEMS syndrome: autologous transplantation, melphalan and dexamethasone, or lenalidomide and dexamethasone? | Leukemia

  https://www.nature.com/articles/s41375-019-0391-2

  MDex therapy included melphalan 10mg/m2+dexamethasone 40mg on days 1-4, one cycle every 28 days for a total of 9 cycles. […] LDex therapy included lenalidomide 10-25mg on days 1-21, dexamethasone 40mg on days 1, 8, 15, 22, one cycle every 28 days for a total of 12 cycles. Prophylaxis of embolization with LDex therapy was by aspirin 100mg qd. […] In conclusion, all three treatments had reasonable responses and survivals in newly diagnosed POEMS syndrome patients. However, ASCT might have better responses and survivals compared with the other two groups, especially for higher risk patients. The treatment should be based on a comprehensive evaluation of the physical and the economic status of the patient, the planned follow-up, and the experience of the physicians and treatment center. ASCT could be recommended for high-risk patients if they are eligible. Lenalidomide maintenance after LDex treatment may be beneficial in term of PFS.

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

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9741379/

  The treatment of POEMS syndrome can be divided into two major categories: targeting the clone by specific anti-plasma cell directed therapy and targeting the symptoms with symptomatic and supportive care. […] The most efficacious strategy is taking the baseline parameters assessing them every visit or every other visit. […] If diagnostic imaging tests (CT scan, MRI, or PET scan) reveal 13 bone lesions with no bone marrow involvement then radiation therapy is the first line of therapy with excellent long-term outcomes. […] In cases of bone marrow involvement or more than three bone lesions, melphalan based conditioning followed by ASCT is considered the first line treatment by many; given the patient is able to tolerate the treatment based on functional status and adequate organs function.

• #1 POEMS syndrome

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

  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%). […] As POEMS syndrome is rare, there are no standard treatments or randomised controlled trials to inform us which treatment is most effective. Management depends on treatment of the underlying plasma cell disorder and may include radiation therapy, chemotherapy or haematopoietic cell transplantation. […] Around 75% of patients have some response to treatment, and VEGF levels (if available) can be used to follow response.

• #1 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 latest evidence suggests that the blood concentration of this cytokine correlates with the activity of the syndrome: VEGF could then be used as a therapeutic target and a marker to monitor response. […] 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. Bevacizumab is a recombinant humanized monoclonal antibody binding to the circulating VEGF-A isoform, thus blocking its interaction with VEGFR. […] The use of this biologic material could then overestimate the in vivo real amount of the cytokine. […] The rapid improvement of the general status allowed the autologous stem-cell transplant and the complete recovery of the patient.

• #1 ABSTRACT

  https://www.ejcrim.com/index.php/EJCRIM/article/download/1241/1875?inline=1

  POEMS syndrome should be suspected in the presence of peripheral polyneuropathy associated with monoclonal gammopathy; diagnostic workup is challenging and delay in treatment is very common. […] There is no consensus on the therapeutic approach, which can include autologous transplantation and chemotherapy. Frequently, these patients present fatigue, weight loss and sarcopenia with reduction of performance status, limiting subsequent therapeutic options. […] Therapeutic options with respect to POEMS syndrome depend on the treatment of the underlying plasma cell disorder. There is no consensual treatment and recommendations are made based on retrospective studies and case reports by adopting some other already existing therapeutics coming from treatment of multiple myeloma, plasmacytoma or amyloidosis. Firstly, it is necessary to evaluate the existence of systemic involvement or the quantity of bone lesions. If there is no medullar infiltration and a limited number of bone lesions, patients should receive radiotherapy. Adjuvant treatment for patients with severe neuropathy can be carried out with corticosteroids or chemotherapy. Patients with medullary involvement or no bone lesions should receive a systemic treatment. The best-response treatment based on evidence is high-dose chemotherapy followed by autologous stem cell transplantation. Autologous cell transplantation seems to be the only effective treatment for POEMS and the first choice for patients without comorbidities. […] When patients cannot be treated with autologous stem cell transplantation, other alternatives can present less successful outcomes: corticosteroids, alkylating agents (lenalidomide, thalidomide or cyclophosphamide).

• #1 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

  Despite the pathogenic importance of this cytokine, the attempts to use it as a marker disease and a therapeutic target are anecdotal and often unsatisfactory. […] Based on the extraordinary results shown in other plasma-cell neoplasms, immunomodulants and proteasome inhibitors, such as lenalidomide and ixazomib, respectively, either alone or in combination with high-dose steroids, and monoclonal antibodies targeting CD38 antigen, such as daratumumab, have been investigated in several clinical trials for patients with POEMS syndrome. […] Meanwhile, further efforts are required to better identify the real role of VEGF and clarify whether it can be used in clinical practice.

• #1 Successful treatment of a patient with POEMS syndrome using thalidomide

  https://www.pulsus.com/scholarly-articles/successful-treatment-of-a-patient-with-poems-syndrome-using-thalidomide.html

  POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) syndrome is a paraneoplastic disorder due to a clonal plasma cell neoplasm. Because the pathogenesis of the disease is unclear, optimal therapeutic management remains unclear. We herein describe a patient with POEMS syndrome treated with thalidomide, which led to a dramatic improvement in her clinical condition. […] Treatment with thalidomide (100 mg/day, increased to 200 mg/day 10 days later for eight months) was initiated plus intravenous methylprednisolone (100 mg/day for 14 consecutive days, repeated every 30 days for two months). […] Thalidomide has been successfully used in the treatment of multiple myeloma, and it is becoming a promising agent for POEMS syndrome. […] Therefore thalidomide, which has anti-angiogenic, anti-inflammatory and immunomodulatory actions, may have controlled abnormally secreted cytokines and contributed to the improvement of POEMS syndrome-related symptoms in the present case.

• #1 Successful treatment of a patient with POEMS syndrome using thalidomide

  https://www.pulsus.com/scholarly-articles/successful-treatment-of-a-patient-with-poems-syndrome-using-thalidomide.html

  The present patient who received eight months of continuous thalidomide therapy did not suffer from worse peripheral neuropathy, which suggested long-term thalidomide treatment is safe for POEMS syndrome patients; however, the safety of long-term usage of thalidomide for POEMS syndrome patients should be evaluated in prospective studies. […] In conclusion, we successfully treated POEMS syndrome complicated by pulmonary arterial hypertension, cardiomegaly and congestive heart failure, suggesting that thalidomide is also an effective therapeutic strategy.

• #1 Anti-BCMA CAR-T cells for treatment of plasma cell dyscrasia: case report on POEMS syndrome and multiple myeloma | Journal of Hematology & Oncology | Full Text

  https://jhoonline.biomedcentral.com/articles/10.1186/s13045-018-0672-7

  POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome still has no standard treatment. […] Our findings demonstrate the anti-BCMA CAR-T cell treatment may be a feasible therapeutic option for patients with POEMS syndrome and RRMM who do not respond well to traditional therapies.

• #1 POEMS syndrome with undetectable M-protein: a case report and literature review | Diagnostic Pathology | Full Text

  https://diagnosticpathology.biomedcentral.com/articles/10.1186/s13000-024-01502-4

  Monoclonal PC dyscrasia (M-protein) while being a mandatory criterion for POEMS syndrome is undetectable in a considerable amount of patients that otherwise demonstrate typical symptoms. […] Therefore, negative results in M-protein and monoclonal PCs arent enough to reject the diagnosis of POEMS syndrome. It is imperative to recognize the variant form of POEMS syndrome. […] Anti-PC therapy has an irreplaceable position in the treatment of POEMS syndrome. For those with systemic disease, the top picks are demonstrated with induction chemotherapy followed up with high-dose melphalan and stem cell rescue if eligible. As for transplant-ineligible patients, lenalidomide combined with dexamethasone remains a preferred treatment option. Additionally, CD38 monoclonal antibodies, B cell maturation antigen-Chimeric antigen receptor T cells (BCMA-CART), and other therapies have also appeared in clinical research. Several studies have revealed that anti-VEGF monoclonal antibody has modest effects on patients with POEMS syndrome.

• #1 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. […] Early diagnosis and active treatment significantly modify the progression of the disease, as well as its overall prognosis. Patients with longer survival and lower disease burden were those who received adjuvant treatment with autologous stem cell transplantation. […] The diagnosis is especially challenging, but with an adequate clinical approach, supported by complementary studies focused on findings that identify the systemic involvement of the disease are enough, as well as the search for elements that allow ruling out the main differential diagnoses, such as chronic inflammatory demyelinating polyneuropathy or light chain amyloidosis. To the extent that the diagnosis is made early, patients benefit more from treatment, since it decreases the progression of the disease and has an impact on survival.

• #2 POEMS syndrome | MedLink Neurology

  https://www.medlink.com/articles/poems-syndrome

  No preventive measures to reduce the risk of POEMS have been identified.

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