Materiały źródłowe

• #1 Signs & Symptoms – Castleman Disease Collaborative Network | CDCN

  https://cdcn.org/castleman-disease/signs-symptoms/

  Castleman disease is a group of rare disorders characterized by lymph node enlargement, specific microscopic changes to the lymph nodes, and a range of symptoms and laboratory findings. […] Castleman disease has a broad spectrum of disease severity, ranging from mild compressive symptoms and fatigue to life-threatening multi-organ failure. Symptoms of Castleman disease often overlap with symptoms of other more common illnesses which makes diagnosis difficult. […] In unicentric Castleman disease (UCD), enlarged lymph nodes appear in only one region of the body. UCD symptoms tend to be mild and occur secondary to compression of surrounding structures by rapidly enlarging lymph nodes. Less commonly, some UCD patients can experience systemic inflammatory symptoms such as fatigue and laboratory abnormalities such as low hemoglobin and elevated C-reactive protein.

• #1 Castleman Disease: Types, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17920-castleman-disease

  Castleman disease symptoms vary depending on the type. You likely wont have symptoms with unicentric Castleman disease. Enlarged lymph nodes may be the only sign. Sometimes, symptoms occur when an enlarged lymph node creates pressure on a nearby organ. […] MCD is more likely to cause noticeable symptoms. In addition to enlarged lymph nodes, signs and symptoms include: Fever, Fatigue (may be a sign of anemia), Night sweats, Nausea and vomiting, Unexplained weight loss, Swelling in your feet, ankles or abdomen, Enlarged spleen (splenomegaly) or liver (hepatomegaly), Numbness in your hands and feet (peripheral neuropathy). […] The prognosis (outlook) is good for most people with UCD. Removing the affected lymph node is often enough to cure it. With treatment, UCD doesnt usually affect life expectancy.

• #1 Castleman Disease | Condition | UAMS Health

  https://uamshealth.com/condition/castleman-disease/

  Castleman disease can cause a variety of symptoms and severity is very broad, ranging from gradual enlargement of lymph nodes with mild symptoms to sudden, intense onset of symptoms due to life-threatening organ dysfunction caused by elevated cytokines such as IL-6. […] Multicentric Castleman disease usually includes widespread chronic swelling of the lymph nodes and sometimes enlargement of the liver and spleen. […] Many people with unicentric Castleman disease have enlarged lymph nodes in one region and experience mild or no symptoms. When symptoms are present, they are usually due to compression of vital structures, such as the trachea, blood vessels or nerves, caused by enlarged lymph nodes. […] Symptoms of Unicentric Castleman Disease may include: A feeling of fullness or pressure in the chest or abdomen that can make breathing or eating difficult, An enlarged lump under the skin in the neck, groin or armpit, Fatigue, Fullness in the face, Weight loss, Cough, Anemia, UCD symptoms can occur secondary to compression of surrounding structures by the enlarged lymph nodes. Less commonly, some UCD patients can experience systemic inflammatory symptoms such as fatigue and laboratory abnormalities such as low hemoglobin and elevated C-reactive protein.

• #1 Castleman Disease: Types, Causes, Symptoms, Diagnosis, and Treatment

  https://www.webmd.com/hiv-aids/castleman-disease

  How you feel depends on which type of Castleman disease you have. With UCD, you may not have symptoms. Or you might notice a bulge from the hard growth in the lymph nodes near your neck or under your arms. […] When UCD causes growths in the lymph nodes in your chest or belly, you may not feel the swelling. But those enlarged areas might bring on other symptoms. A growth in a lymph node in your chest could lead to: Trouble breathing, Wheezing or coughing, Feeling of fullness in your chest. […] If the lymph node growth is in your belly, you may have: Trouble eating, Feeling of fullness in your stomach. […] If you have MCD, you may have some of the same symptoms as UCD. You might also notice things like: Fatigue, Fever, Loss of appetite, Rashes, Sweating, especially during the night, Weak or numb hands or feet, Weight loss.

• #1 Signs & Symptoms – Castleman Disease Collaborative Network | CDCN

  https://cdcn.org/castleman-disease/signs-symptoms/

  In multicentric Castleman disease (MCD), enlarged lymph nodes appear in multiple regions of the body. Symptoms occur due to a systemic hyperinflammatory response and tend to be more severe than UCD symptoms. Flu-like symptoms (fever, night sweats, loss of appetite, nausea, and vomiting). Edema (swelling), ascites (fluid accumulation in the abdomen), and/or other symptoms of fluid accumulation. Unintentional weight loss. Weakness, fatigue. Enlarged liver or spleen. Kidney dysfunction. Peripheral neuropathy (numbness in the hands and feet). Cherry hemangioma skin rash.

• #1 Castleman Disease | Condition | UAMS Health

  https://uamshealth.com/condition/castleman-disease/

  Multicentric Castleman Disease usually includes widespread enlargement of the lymph nodes. The enlarged nodes can be in the chest or abdomen, groin or the underarm area. When present on the sides of the neck, they can often be seen or felt as lumps under the skin. […] Multicentric Castleman disease can also affect lymphoid tissue of internal organs, such as the liver or spleen, causing them to enlarge. Enlarged organs might be seen or felt as masses under either side of the rib cage. They can cause a sense of fullness or pain in the abdomen and can interfere with eating. […] Patients with MCD have enlarged lymph nodes in multiple regions and more severe symptoms. Signs and symptoms of multicentric Castleman disease (MCD) include: Flu-like symptoms: fever, fatigue, weight loss, night sweats, loss of appetite, nausea and vomiting, Abnormally large lymph nodes, typically in the neck, armpit, collarbone, and groin, Enlarged spleen or liver, Cherry hemangioma, Edema (swelling), ascites (fluid accumulation in the abdomen), and/or other symptoms of fluid accumulation, Peripheral neuropathy (numbness in the hands and feet), Elevated inflammatory markers (C-reactive protein, erythrocyte sedimentation rate), Kidney dysfunction, Low albumin, Anemia (low amount of red blood cells), Low or high platelet counts, Elevated immunoglobulins or gamma globulins.

• #1 Overview – Castleman Disease Collaborative Network | CDCN

  https://cdcn.org/castleman-disease/overview/

  iMCD also has important differences in symptoms, disease course, and treatment from POEMS-associated MCD and HHV-8-associated MCD. […] iMCD with TAFRO syndrome (iMCD-TAFRO) is characterized by acute episodes of Thrombocytopenia, Anasarca, Fever, Renal dysfunction or myelofibrosis, and Organomegaly. […] iMCD with idiopathic plasmacytic lymphadenopathy (iMCD-IPL) is characterized by thrombocytosis, hypergammaglobulinemia, and a more chronic disease course.

• #1 Castleman disease – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/castleman-disease/symptoms-causes/syc-20543017

  Many people with unicentric Castleman disease don’t notice any signs or symptoms. The enlarged lymph node may be found during a physical exam or an imaging test for a different problem. […] Some people with unicentric Castleman disease might have signs and symptoms that are more often seen in multicentric Castleman disease. These may include: Fever. Weight loss that happens without trying. Fatigue. Night sweats. Swelling. Enlarged liver or spleen. […] Symptoms of the more serious form of idiopathic MCD called iMCD-TAFRO are: Low platelet count, also called thrombocytopenia. Swelling and fluid in the body, known as anasarca. Fever or a higher level of C-reactive protein, a marker of inflammation. Reticulin fibrosis, which is checked by taking a sample of bone marrow. Organ swelling, also called organomegaly.

• #1 Idiopathic multicentric Castleman disease – CAP TODAY

  https://www.captodayonline.com/idiopathic-multicentric-castleman-disease/

  Idiopathic multicentric Castleman disease, which is driven by a cytokine storm with an unknown cause, is a difficult diagnosis and one that’s often delayed, owing to the disease’s rarity and nonspecific symptoms. […] The symptoms can mimic infections, autoimmune diseases, and malignancies. The patients can present variably. Some are asymptomatic with a few laboratory abnormalities; others have some combination of fatigue, edema, weight loss, night sweats, ascites, fever, or enlarged liver or spleen. Some patients present with multiorgan failure. […] TAFRO is an iMCD subtype that has a severe or aggressive presentation, so it’s on the far end of the spectrum. TAFRO patients present with thrombocytopenia, anasarca or ascites, reticulin fibrosis in the bone marrow, renal dysfunction, and organomegaly. Patients with TAFRO have severe disease and a higher likelihood of death than patients presenting without TAFRO.

• #1 Castleman Disease: Types, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17920-castleman-disease

  The outlook for people with MCD depends on the type of MCD and how serious it is. Some people have intense symptom flare-ups that can be life-threatening. Others have more chronic symptoms. Sixty-five percent to 75% of people diagnosed with MCD are still alive five years later. With breakthrough therapies, theres hope that the outlook will continue to improve.

• #1 Unicentric Castleman disease – Wikipedia

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

  Complete surgical removal of the enlarged lymph node or region of lymph nodes is considered the gold standard treatment for UCD and is typically curative, with resolution of symptoms and lab abnormalities attributable to the disease. […] Most people with UCD who undergo complete surgical resection of enlarged lymph nodes achieve long-term disease-free survival, with an observed ten-year mortality of 4% in the largest case series to date.

• #1 Castleman disease – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/castleman-disease/symptoms-causes/syc-20543017

  Idiopathic multicentric Castleman disease can rapidly get worse to involve life-threatening problems with organ function. This requires critical care with a machine that helps with breathing, called a ventilator, and treatments that help with organs function, such as dialysis and transfusions. […] HHV-8-positive multicentric Castleman disease may involve life-threatening infections and organ failure. People who also have HIV/AIDS generally have worse outcomes.

• #1 Idiopathic multicentric Castleman disease – CAP TODAY

  https://www.captodayonline.com/idiopathic-multicentric-castleman-disease/

  If not treated appropriately, patients with iMCD have a 35 percent mortality rate within five years of diagnosis. The disease is progressive and characterized by flares, and each time they come back, they’ll be a little bit worse. […] If left untreated, eventually patients will have multiorgan dysfunction and death.

• #1

  https://haematologica.org/article/view/haematol.2023.283603

  Idiopathic multicentric Castleman disease (iMCD) is a rare hematologic disorder with heterogeneous presentations ranging from moderate constitutional symptoms to life-threatening multiorgan system involvement. […] There are vastly different clinical subtypes, with some patients demonstrating thrombocytopenia, anasarca, fever/elevated C-reactive protein, reticulin fibrosis/renal failure, and organomegaly (TAFRO) and others having milder/more moderate symptoms with potential for severe disease (not otherwise specified, NOS). […] We found that iMCD-TAFRO patients face a significant hospitalization burden, requiring more time in the hospital than iMCD-NOS patients during the year surrounding diagnosis (median [interquartile range]: 36 [18-61] days vs. 0 [0-4] days; P0.001). […] In addition, we found life-sustaining interventions, such as mechanical ventilation (17%) and dialysis (27%), were required among iMCD patients, predominantly those with iMCD-TAFRO.

• #1

  https://haematologica.org/article/view/haematol.2023.283603

  iMCD-NOS patients, however, spent a significantly greater proportion of time following disease onset in a state of disease flare (median 52.3% vs. 18.9%; P=0.004). […] Lastly, we observed severe iMCD-related morbidities, such as acute renal failure, sepsis and pneumonia, among others, arising after iMCD diagnosis, impairing the patients quality of life. […] While the pathophysiology of the disease remains poorly understood, existing data have implicated interleukin 6 and the mTOR and JAK-STAT pathways. […] As with other inflammatory disorders, iMCD is characterized by constitutional symptoms and systemic inflammation that can lead to multiorgan failure or death. […] A large majority of patients presented with fluid retention (84%), splenomegaly (72%), and/or hepatomegaly (60%). […] We examined the number of hospitalized days due to iMCD symptoms as another measure of disease burden.

• #1

  https://haematologica.org/article/view/haematol.2023.283603

  Following the iMCD diagnosis, 48% (n=49) of the cohort developed acute renal failure, 15.7% (n=16) developed chronic kidney disease, 13.7% (n=14) developed iron deficiency anemia, 10.7% (n=11) developed pneumonia, 6.9% (n=7) developed sepsis, and 6.9% (n=7) developed thrombotic microangiopathy. […] This study found that iMCD patients experienced a greater degree of hospitalization than the general population experiences in a given year, with this being primarily due to the increased rates of hospitalization among iMCD-TAFRO patients.

• #1

  https://haematologica.org/article/view/haematol.2023.283603

  We found that TAFRO patients spent significantly more days in hospital than did NOS patients. […] This demonstrates that compared to the general population, patients with iMCD require substantially greater use of the healthcare system and frequently need to be admitted to hospital. […] Considering the degree of hospitalization burden, we next investigated the distribution of organ system involvement among iMCD patients 365 days from diagnosis. […] We found that 27 (26.5%) patients required dialysis and 17 (16.7%) patients required a ventilator during at least one iMCD-related hospitalization. […] We also quantified the proportion of time patients spent in a state of flare. […] These data suggest that while TAFRO patients experience a greater degree of organ failure and more hospital interventions, NOS patients experience a longer continuation of milder, chronic symptoms.

• #1 Castleman Disease: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/2219018-overview

  Patients experience atypical CD-like lymphoproliferation, which occurs along with systemic inflammatory signs and symptoms (fevers, weight loss, fatigue, night sweats), abnormal blood cell counts, and multiple organ system dysfunction. […] The prognosis of Castleman disease varies, depending on the subtype. […] The prognosis for iMCD patients is worse than for patients with HHV-8associated MCD. […] A study of the ACCELERATE registry found that patients with HHV-8negative (idiopathic) MCD may experience substantial disease burden and impaired quality of life. Severe disease-related morbidity may include acute kidney, sepsis, and pneumonia, among others.

• #1 Castleman Disease Clinical Presentation: History and Physical Examination

  https://emedicine.medscape.com/article/2219018-clinical

  Idiopathic MCD involves multicentric lymphadenopathy with characteristic „CD-like” lymph node histopathology and a number of signs/symptoms and laboratory features, as defined by the 2017 International Consensus Diagnostic Criteria of idiopathic MCD, which may progress or remit/relapse over time: Elevated C-reactive protein (CRP) level and/or erythrocyte sedimentation rate (ESR), Anemia, Thrombocytopenia or thrombocytosis, Hypoalbuminemia, Kidney dysfunction and/or proteinuria, Polyclonal hypergammaglobulinemia, Flulike illness (night sweats, fever, weight loss, fatigue), Large liver and/or spleen, Fluid accumulation (edema, anasarca, ascites, pleural effusion), Eruptive cherry hemangiomatosis or violaceous papules, Lymphocytic interstitial pneumonitis. […] The flulike signs and symptoms and acute-phase reaction, with elevation of the ESR and CRP level, elevated fibrinogen, thrombocytosis, and hypergammaglobinemia in HHV-8associated MCD and idiopathic MCD, are believed to be driven by overproduction of interleukin-6 (IL-6).

• #1 Castleman-Kojima Disease: Symptoms, Causes, and Treatment Guide – The Kingsley Clinic

  https://thekingsleyclinic.com/resources/castleman-kojima-disease-symptoms-causes-and-treatment-guide/

  Fever occurs in 40–60% of patients with Castleman-Kojima disease and is often one of the earliest symptoms. […] Fatigue affects 70–80% of patients and can significantly impact quality of life. […] Unintentional weight loss is reported in 50–60% of cases. […] Enlarged lymph nodes, or lymphadenopathy, are present in nearly all patients with Castleman-Kojima disease. […] Night sweats affect 40–50% of patients and are often described as episodes so intense that they require a change of clothes or bedding. […] Anemia is present in 60–70% of patients and is characterized by a reduced number of red blood cells or lower hemoglobin levels. […] Thrombocytopenia, or a low platelet count, affects 30–40% of patients. […] Hypergammaglobulinemia, an elevated level of antibodies in the blood, occurs in 50–60% of cases.

• #1 Recognizing Symptoms of iMCD | SYLVANT® (siltuximab)

  https://sylvant.com/understanding-imcd/symptoms-of-imcd/

  Here are a few iMCD symptoms that will help you better understand the disease and what to look for: […] Swollen lymph nodes are one of your body’s natural reactions to illness or infection […] Fatigue is defined as an overall feeling of tiredness or having low energy […] Excessive sweating while you sleep […] An increase in normal body temperature, a key sign that your immune system has been activated […] A condition in which a person has a low red blood cell count and might feel tired […] Malaise is a general feeling of discomfort, illness, or lack of well-being […] Unexpected change in weight can be a sign of an underlying medical condition […] The symptoms that led up to my iMCD diagnosis included visibly swollen lymph nodes, fatigue, night sweats, headaches, and nausea. […] Getting to an iMCD diagnosis may be difficult—symptoms of iMCD can range from mild to severe and look like other disorders.

• #1 Castleman-Kojima Disease: Symptoms, Causes, and Treatment Guide – The Kingsley Clinic

  https://thekingsleyclinic.com/resources/castleman-kojima-disease-symptoms-causes-and-treatment-guide/

  Hepatomegaly, or an enlarged liver, is found in 20–30% of patients. […] Splenomegaly, or an enlarged spleen, affects 30–40% of patients. […] The progression of Castleman-Kojima disease depends on its subtype. UCD may not produce significant symptoms, while MCD can lead to widespread inflammation and complications, including organ dysfunction.

• #1 Idiopathic multicentric Castleman disease and associated autoimmune and autoinflammatory conditions: practical guidance for diagnosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10070070/

  Many clinical manifestations of the iMCD subtypes, despite some overlapping features, are different from those of iMCD-NOS and TAFRO. iMCD-TAFRO comprises an aggressive clinical subtype of iMCD involving thrombocytopenia, anasarca, reticulin fibrosis, renal dysfunction and organomegaly. […] Anaemia is a common symptom of iMCD. In the largest series, anaemia was reported in 79 of 91 patients in whom haemoglobin was measured. […] Renal dysfunction or proteinuria is frequently observed in MCD, mainly in the mixed or plasma cell subtype, with very heterogeneous clinical and histological findings. […] Demyelinating peripheral neuropathy is frequently observed with CD. In fact, the presence of peripheral neuropathy is a feature that supports the iMCD diagnosis, although it is not necessary for the diagnosis.

• #1 Idiopathic multicentric Castleman disease and associated autoimmune and autoinflammatory conditions: practical guidance for diagnosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10070070/

  The severity of peripheral neuropathy is less in CD patients with peripheral neuropathy but not concurrent POEMS, followed by POEMS-MCD and worst in classic POEMS without MCD. […] The coexistence of CD, inflammatory disorders and autoantibodies, in the same patient, including quite specific autoantibodies such as anti-Sjgren-syndrome-related antigen A (SSA) or anti-centromere, may be explained because they likely share some pathophysiology features.

• #1 Castleman Disease Clinical Presentation: History and Physical Examination

  https://emedicine.medscape.com/article/2219018-clinical

  HHV-8associated multicentric Castleman disease (HHV-8associated MCD) involves multicentric lymphadenopathy with characteristic „CD-like” lymph node histopathology and/or the following systemic manifestations, although some cases (10%) are asymptomatic: Flulike illness (fever, night sweats that soak the sheets, weight loss, loss of appetite, weakness, fatigue), Shortness of breath, cough, Nausea and vomiting, Numbness and weakness (neuropathy), Leg edema, Skin rashes, Hemangiomata, Pemphigus, Kaposi sarcoma. […] For idiopathic multicentric Castleman disease, according to the Castleman Disease Collaborative Network (CDCN), at least 2 of following must be present for it to be classified as severe: Eastern Cooperative Oncology Group (ECOG) performance status 2 or above, Stage IV kidney dysfunction (estimated glomerular filtration rate 30 mL/minute/1.73m2, creatinine 3 mg/dL), Anasarca and/or ascites, pleural effusion, or pericardial effusion, Hemoglobin 8 g/dL, Pulmonary involvement/interstitial pneumonitis with dyspnea.

• #1 Treatment guidelines (disease severity classification / disease activity index) |　Castleman disease, TAFRO, and related disease research group

  https://castleman.jp/en/introduction.html

  Castleman disease can present with various clinical symptoms of different degrees of severity. Frequent symptoms include low- to moderate-grade fever, general malaise, easy fatigability, weight loss, night sweats, and superficial lymphadenopathy. Some cases manifest with skin lesions (flat or slightly elevated brown or dark red eruptions, pemphigoids, xanthoma, atopic dermatitis, or hemangiomas), abdominal distension, edema, shortness of breath, dyspnea, and hemorrhagic tendency. Castleman disease can be associated with vascular events such as cerebral infarction, and peripheral neuropathy. […] In addition to lymphadenopathy, imaging examinations may reveal hepatosplenomegaly, ascites, pleural effusion, and pulmonary interstitial shadows. […] In some cases, renal dysfunction, interstitial pulmonary lesions, pulmonary hypertension, dilated cardiomyopathy, autoimmune thrombocytopenia, autoimmune hemolytic anemia, endocrinopathy (such as hypothyroidism), and/or AA-amyloidosis accompany the disease.

• #1 Castleman Disease Clinical Presentation: History and Physical Examination

  https://emedicine.medscape.com/article/2219018-clinical

  The four subtypes of Castleman disease can each cause a variety of signs and symptoms. […] Unicentric Castleman disease (UCD) tends to have milder symptoms and rarely affects vital organs such as the liver, kidneys, and bone marrow but may present as follows: Enlargement of a single lymph node or single region of lymph nodes (eg, right cervical chain), possibly with resultant compressive symptoms. […] The presentation of multicentric Castleman disease spans a wide spectrum of severity, from mild symptoms to life-threatening organ failure. […] The National Comprehensive Cancer Network (NCCN) criteria for active disease include fever, C-reactive protein 20 mg/L in the absence of other causes, and 3 or more of the following: Peripheral lymphadenopathy, Splenomegaly, Edema, Pleural effusion, Ascites, Cough, Nasal obstruction, Xerostomia, Rash, Central nervous system symptoms, Jaundice, Autoimmune hemolytic anemia.

• #1 Castleman Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK576394/

  Elevated fibrinogen levels cause deep vein thrombosis and thromboembolic disorders, high hepcidin levels cause anemia, increased VEGF levels result in angiogenesis and vascular permeability, which along with hypoalbuminemia induces edema, ascites, pleural and pericardial effusions, or even generalized anasarca. […] In extreme cases of iMCD, multiple organ failure with renal insufficiency can ensue, often resulting in death. […] UCD being able to be eliminated with surgical resection has a good prognosis. However, since multiple systemic disorders involve MCD and sometimes concomitant diseases like HIV and HHV-8 can also be present, specific chemotherapy regimens have not been assigned, and better treatment methods still need to be explored. […] The survival rate is significant in patients without these complications. Therefore, early diagnosis for these manifestations is necessary for tailoring appropriate therapy options, leading to a better prognosis.

• #1 Treatment guidelines (disease severity classification / disease activity index) |　Castleman disease, TAFRO, and related disease research group

  https://castleman.jp/en/introduction.html

  Elevation of serum IgG4 levels or increased IgG4 positive cells in the lymph nodes can be observed, not only in IgG4-related disease, but also in Castleman disease. Presence of fever, microcytic anemia, thrombocytosis, and elevations of serum CRP, which are caused by overproduction of IL-6, are signs supporting the diagnosis of Castleman disease rather than IgG4-related disease. […] The research group has proposed a system to classify the severity of Castleman disease into three stages of mild, moderate, and severe depending mainly on organ disorders related to life prognosis.

• #1 Castleman Disease | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/castleman-disease

  Castleman disease is a rare immune dysregulation disorder where an overgrowth of cells in your childs lymph nodes causes them to be enlarged and sometimes also causes fevers or other signs of systemic inflammation (when the immune system is constantly defending the body). […] Patients with MCD usually have lymph node swelling as well as flu-like symptoms (e.g. fever, weight loss, night sweats). […] If your child has Castleman disease, you may notice symptoms like: Fever, Unintended weight loss, Fatigue, Night sweats, Raised lumps under the skin, especially in the neck, underarm, collarbone or groin area. […] More severe symptoms of Castleman disease can include flu-like symptoms, a feeling of fullness in the chest or abdomen that doesnt go away, or an enlarged liver or spleen. […] Multicentric Castleman disease can be life-threatening and lead to severe infections or organ failure.

• #1 Idiopathic multicentric Castleman disease in children: a single-center retrospective analysis | BMC Pediatrics | Full Text

  https://bmcpediatr.biomedcentral.com/articles/10.1186/s12887-024-05347-0

  Children with multicentric CD often have systemic symptoms, lymph node enlargement and related compression symptoms are the most common manifestations, followed by fever, malaise and other systemic symptoms. […] The initial symptoms of the children included fever (5/9 cases), lymph node enlargement and its resulting compression symptoms (such as abdominal pain, chest pain, etc.) (6/9 cases), fatigue (3/9 cases), and changes in blood count (1/9 cases). 8 cases had systemic symptoms, including fever (5/8 cases), vomiting (2/8 cases), and multiple serous cavity effusion (3/8 cases) being more common. Other systemic symptoms included cough, fatigue, etc. […] Systemic inflammatory response is also common in iMCD patients, often accompanied by fever, elevated inflammatory indicators, and multi-organ involvement.

• #1 The French paediatric cohort of Castleman disease: a retrospective report of 23 patients | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-1345-5

  We identified 23 patients (12 girls) with a diagnosis of UCD (n=17) and MCD (n=6) between 1994 and 2018. The mean age at first symptoms was 11.47 4.23years for UCD and 8.3 3.4years for MCD. The mean diagnosis delay was 8.16 10.32months for UCD and 5.16 5.81years for MCD. In UCD, the initial symptoms were isolated lymph nodes (n=10) or lymph node associated with other symptoms (n=7); fever was present in 3 patients. Five patients with MCD presented fever. […] For patients with MCD, the mean age at the first symptoms was 8.33.4years (range 2.813). They presented fever (5/6; 83.3%), abdominal lymph nodes (5/6; 83.3%), failure to thrive (3/6; 50%), hepatomegaly and/or splenomegaly (3/6; 50%), arthralgia (2/6; 33.3%), abdominal pain (2/6; 33.3%), fatigue (2/6; 33.3%), facial oedema (1/6;16.7%), isolated lymphadenopathy (1/6; 16.7%), rash on the trunk (1/6; 16.7%), vascular hepatopathy with oesophageal varicose veins (1/6; 16.7%), diarrhoea (1/6; 16.7%) and cholestasis (1/6; 16.7%).

• #1 Idiopathic multicentric Castleman disease in children: a single-center retrospective analysis | BMC Pediatrics | Full Text

  https://bmcpediatr.biomedcentral.com/articles/10.1186/s12887-024-05347-0

  Pediatric iMCD patients are more likely to experience disease progression and recurrence. In this article, 8 out of 9 patients experienced varying degrees of disease recurrence or progression during diagnosis and treatment. However, the overall prognosis is generally good, with few deaths occurring after receiving reasonable treatment.

• #1 Unicentric Castleman disease – Wikipedia

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

  People with unicentric Castleman disease (UCD) have an enlarged lymph node or multiple enlarged lymph nodes in a single lymph node region. […] Symptoms are typically mild, abnormalities on blood tests are uncommon, organ dysfunction is uncommon, and surgical treatment is curative in the majority of patients. […] In approximately half of patients, UCD presents without symptoms; however, people with the disease may experience enlarged lymph nodes in a single lymph node region or report symptoms related to compression of neighboring structures by enlarged lymph nodes, such as difficulty breathing and pain or pressure in the abdomen or chest. […] Systemic symptoms (fever, night sweats, weight loss, fatigue) are seen in up to half of UCD patients. […] Laboratory testing is typically normal, including blood counts, metabolic tests, and inflammatory markers; however, in some people with UCD, laboratory testing may show abnormalities more commonly seen in HHV-8-associated MCD or iMCD.

• #1 The French paediatric cohort of Castleman disease: a retrospective report of 23 patients | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-1345-5

  The main reasons for the diagnostic delay in CD are probably the lack of specificity of calling symptoms combined with little awareness of this condition among paediatricians as well as insufficient dialogue with pathologists. Unfortunately, the diagnostic delay has deleterious consequences such as increased morbidity due to chronic inflammation in children, particularly growth retardation, and significant burden related to useless explorations and untimely treatments.

• #2 Overview – Castleman Disease Collaborative Network | CDCN

  https://cdcn.org/castleman-disease/overview/

  Castleman disease (CD) describes a group of rare disorders that involve enlarged lymph nodes and a broad range of inflammatory symptoms and laboratory abnormalities. […] The symptoms, causes, and treatments vary greatly for each subtype of CD. […] UCD tends to have milder symptoms and rarely affects vital organs such as the liver, kidneys, and bone marrow. […] The three subtypes of MCD are characterized by lymph node enlargement in multiple regions of the body with characteristic microscopic features, flu-like symptoms, and organ dysfunction due to excessive cytokines or inflammatory proteins. […] In HHV-8+MCD, uncontrolled infection with HHV-8 causes the immune system to produce excessive cytokines that lead to MCD. […] Though iMCD patients experience excessive cytokine production and a cytokine storm, the cause is unknown.

• #2 Castleman Disease Causes, Symptoms, Risk Factors, Diagnosis and Treatment

  https://www.prepladder.com/neet-pg-study-material/pathology/castleman-disease-causes-symptoms-risk-factors-diagnosis-and-treatment

  Many people with unicentric Castleman disease have no symptoms at all. The enlarged lymph node may be discovered during a physical examination or an imaging test for an unrelated condition. […] Some cases of unicentric Castleman disease may present with signs and symptoms that are more common in cases of multicentric Castleman disease, including: High temperature. Unintended reduction in weight. Tiredness. Sweating at night. An enlarged liver or spleen. Swollen lymph nodes associated with multicentric Castleman disease are most commonly found in the neck, collarbone, underarm, and groin areas. […] Patients with unicentric Castleman disease usually make a full recovery when the affected lymph node is removed. Infections that may be deadly or organ failure are possible outcomes of multicentric Castleman disease. Those who additionally have HIV/AIDS usually experience the worst outcomes.

• #2 Unicentric Castleman Disease (UCD) – Rare Awareness Rare Education

  https://rareportal.org.au/rare-disease/unicentric-castleman-disease-ucd/

  Symptoms of unicentric Castleman disease (UCD) can vary between individuals. People with UCD usually experience no symptoms. The affected lymph node/s generally appear as a non-painful lump. Occasionally mild fatigue, aches and pains, and mild anaemia can occur. UCD can cause more severe symptoms when the lymph nodes grow in a way that it physically impacts on nearby organs, blood flow or nerves. The symptoms vary depending on what the enlarged lymph nodes impact. […] Please speak to your medical team to learn more about the symptoms and complications of unicentric Castleman disease.

• #2 What Is Multicentric Castleman Disease?

  https://www.healthline.com/health/multicentric-castleman-disease

  Enlarged lymph nodes are one of the main symptoms of multicentric Castleman disease. You may also have an enlarged spleen, an enlarged liver, or both, depending on how far the disease has progressed. […] Other common symptoms may include: fatigue, fever, nausea and vomiting, numbness in your hands and feet, swelling in your abdomen, swelling in your lower legs, unexplained weight loss. […] Multicentric Castleman disease can cause flu-like symptoms and raise the risk of cancer or serious infections. Treatment depends on the type and severity of the disease, so getting a timely diagnosis is vital to improving your odds of a healthy outcome. […] If treatment begins early enough, the outlook for someone with multicentric Castleman disease is generally encouraging. […] Castleman disease can be a life threatening disease without prompt and effective treatment. Potential complications of the disease include severe damage to multiple organs and cancer.

• #2 Pathology Outlines – Castleman disease

  https://www.pathologyoutlines.com/topic/lymphnodescastleman.html

  UCD: asymptomatic or an enlarging lymph node or mass; secondary symptoms related to the mass (compression or pain) (Blood 2020;135:1353) […] MCD: all subtypes of MCD show systemic inflammatory manifestations including fever, weight loss, anasarca, generalized lymphadenopathy and hepatomegaly […] Anemia, hypoalbuminemia, cytopenias and elevated inflammatory markers are common (Blood Adv 2021;5:1660) […] MCD POEMS: a syndrome characterized by peripheral neuropathy, organomegaly, skin changes and monoclonal paraproteins occurs in some patients with MCD (Blood 1994;83:2587) […] MCD TAFRO: another syndrome characterized by thrombocytopenia, ascites, fever, reticulin fibrosis and organomegaly usually in patients with normal immunoglobulin levels and mixed or hyaline vascular histology (Sci Rep 2017;7:42316) […] iMCD, NOS: shows elevated platelet counts and immunoglobulin levels and plasmacytic histology (Blood 2020;135:1353)

• #2 Castleman disease – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/castleman-disease/symptoms-causes/syc-20543017

  Many people with unicentric Castleman disease don’t notice any signs or symptoms. The enlarged lymph node may be found during a physical exam or an imaging test for a different problem. […] Some people with unicentric Castleman disease might have signs and symptoms that are more often seen in multicentric Castleman disease. These may include: Fever. Weight loss that happens without trying. Fatigue. Night sweats. Swelling. Enlarged liver or spleen. […] Symptoms of the more serious form of idiopathic MCD called iMCD-TAFRO are: Low platelet count, also called thrombocytopenia. Swelling and fluid in the body, known as anasarca. Fever or a higher level of C-reactive protein, a marker of inflammation. Reticulin fibrosis, which is checked by taking a sample of bone marrow. Organ swelling, also called organomegaly.

• #2

  https://link.springer.com/article/10.1007/s00431-023-05235-2

  After a median follow-up of 4.08 years (IQR: 1.63, 6.08), all patients had survived and none had developed relapse or progression, 10/16 (63%) were in complete remission, and 6/16 (38%) had stable lymphadenopathy. […] The prognosis of CD is more favorable in children than adults, and as expected, the progression-free survival rate in our study was 100%.

• #2 Unicentric Castleman disease – Wikipedia

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

  Complete surgical removal of the enlarged lymph node or region of lymph nodes is considered the gold standard treatment for UCD and is typically curative, with resolution of symptoms and lab abnormalities attributable to the disease. […] Most people with UCD who undergo complete surgical resection of enlarged lymph nodes achieve long-term disease-free survival, with an observed ten-year mortality of 4% in the largest case series to date.

• #2 Idiopathic multicentric Castleman disease – CAP TODAY

  https://www.captodayonline.com/idiopathic-multicentric-castleman-disease/

  If not treated appropriately, patients with iMCD have a 35 percent mortality rate within five years of diagnosis. The disease is progressive and characterized by flares, and each time they come back, they’ll be a little bit worse. […] If left untreated, eventually patients will have multiorgan dysfunction and death.

• #2 Castleman disease // Middlesex Health

  https://middlesexhealth.org/6D45E88A-F751-989D-073B9A57D2251060

  Many people with unicentric Castleman disease don’t notice any signs or symptoms. The enlarged lymph node may be found during a physical exam or an imaging test for a different problem. […] Some people with unicentric Castleman disease might have signs and symptoms that are more often seen in multicentric Castleman disease. These may include: […] If you notice an enlarged lymph node on the side of your neck or in your underarm, collarbone or groin area, talk with your healthcare professional. Also call your care team if you have a lasting feeling of fullness in your chest or abdomen, fever, fatigue, or weight loss that you can’t explain. […] Idiopathic multicentric Castleman disease can rapidly get worse to involve life-threatening problems with organ function. This requires critical care with a machine that helps with breathing, called a ventilator, and treatments that help with organs function, such as dialysis and transfusions. […] HHV-8-positive multicentric Castleman disease may involve life-threatening infections and organ failure. People who also have HIV/AIDS generally have worse outcomes.

• #2

  https://haematologica.org/article/view/haematol.2023.283603

  iMCD-NOS patients, however, spent a significantly greater proportion of time following disease onset in a state of disease flare (median 52.3% vs. 18.9%; P=0.004). […] Lastly, we observed severe iMCD-related morbidities, such as acute renal failure, sepsis and pneumonia, among others, arising after iMCD diagnosis, impairing the patients quality of life. […] While the pathophysiology of the disease remains poorly understood, existing data have implicated interleukin 6 and the mTOR and JAK-STAT pathways. […] As with other inflammatory disorders, iMCD is characterized by constitutional symptoms and systemic inflammation that can lead to multiorgan failure or death. […] A large majority of patients presented with fluid retention (84%), splenomegaly (72%), and/or hepatomegaly (60%). […] We examined the number of hospitalized days due to iMCD symptoms as another measure of disease burden.

• #2

  https://haematologica.org/article/view/haematol.2023.283603

  We found that TAFRO patients spent significantly more days in hospital than did NOS patients. […] This demonstrates that compared to the general population, patients with iMCD require substantially greater use of the healthcare system and frequently need to be admitted to hospital. […] Considering the degree of hospitalization burden, we next investigated the distribution of organ system involvement among iMCD patients 365 days from diagnosis. […] We found that 27 (26.5%) patients required dialysis and 17 (16.7%) patients required a ventilator during at least one iMCD-related hospitalization. […] We also quantified the proportion of time patients spent in a state of flare. […] These data suggest that while TAFRO patients experience a greater degree of organ failure and more hospital interventions, NOS patients experience a longer continuation of milder, chronic symptoms.

• #2 Castleman disease | City of Hope

  https://www.cancercenter.com/risk-factors/castleman-disease

  Castleman disease (CD) is a rare condition that affects the lymph nodes and related tissues. Its not considered cancer but may act like cancer, and it may develop into lymphoma. While Castleman disease has a wide range of symptoms, the primary sign is enlarged lymph nodes that share a similar appearance when viewed under a microscope. […] In patients with Castleman disease, these cells may become overactive and produce too many messengers, particularly Interleukin-6 (IL-6). Too much IL-6 may lead to flu-like symptoms such as fatigue, night sweats, nausea and weight loss, lymph node enlargement, and issues with vital organs, including the liver, kidneys and bone marrow. Problems with these internal organs may lead to fluid gain, confusion, bruising and bleeding. […] While symptoms vary from person to person, its common to experience: Abdominal pain, Abnormally high amounts of certain immune factors in the blood (hypergammaglobulinemia), Anemia, Elevated C-reactive protein level, Excessive sweating, Fatigue, Fever, Night sweats, Skin rash, Weight loss.

• #2 Castleman Disease | Condition | UAMS Health

  https://uamshealth.com/condition/castleman-disease/

  Multicentric Castleman Disease usually includes widespread enlargement of the lymph nodes. The enlarged nodes can be in the chest or abdomen, groin or the underarm area. When present on the sides of the neck, they can often be seen or felt as lumps under the skin. […] Multicentric Castleman disease can also affect lymphoid tissue of internal organs, such as the liver or spleen, causing them to enlarge. Enlarged organs might be seen or felt as masses under either side of the rib cage. They can cause a sense of fullness or pain in the abdomen and can interfere with eating. […] Patients with MCD have enlarged lymph nodes in multiple regions and more severe symptoms. Signs and symptoms of multicentric Castleman disease (MCD) include: Flu-like symptoms: fever, fatigue, weight loss, night sweats, loss of appetite, nausea and vomiting, Abnormally large lymph nodes, typically in the neck, armpit, collarbone, and groin, Enlarged spleen or liver, Cherry hemangioma, Edema (swelling), ascites (fluid accumulation in the abdomen), and/or other symptoms of fluid accumulation, Peripheral neuropathy (numbness in the hands and feet), Elevated inflammatory markers (C-reactive protein, erythrocyte sedimentation rate), Kidney dysfunction, Low albumin, Anemia (low amount of red blood cells), Low or high platelet counts, Elevated immunoglobulins or gamma globulins.

• #2 What Is Multicentric Castleman Disease? Treatment, Radiology, Symptoms

  https://anavara.com/treatment/castleman-disease

  Some people with unicentric Castleman disease might experience signs and symptoms more common to multicentric Castleman disease, which may include: Fever Unintended weight loss Fatigue Night sweats Nausea Enlarged liver or spleen. […] The enlarged lymph nodes associated with multicentric Castleman disease are most commonly located in the neck, collarbone, underarm and groin areas.

• #2 Castleman Disease Clinical Presentation: History and Physical Examination

  https://emedicine.medscape.com/article/2219018-clinical

  Idiopathic MCD involves multicentric lymphadenopathy with characteristic „CD-like” lymph node histopathology and a number of signs/symptoms and laboratory features, as defined by the 2017 International Consensus Diagnostic Criteria of idiopathic MCD, which may progress or remit/relapse over time: Elevated C-reactive protein (CRP) level and/or erythrocyte sedimentation rate (ESR), Anemia, Thrombocytopenia or thrombocytosis, Hypoalbuminemia, Kidney dysfunction and/or proteinuria, Polyclonal hypergammaglobulinemia, Flulike illness (night sweats, fever, weight loss, fatigue), Large liver and/or spleen, Fluid accumulation (edema, anasarca, ascites, pleural effusion), Eruptive cherry hemangiomatosis or violaceous papules, Lymphocytic interstitial pneumonitis. […] The flulike signs and symptoms and acute-phase reaction, with elevation of the ESR and CRP level, elevated fibrinogen, thrombocytosis, and hypergammaglobinemia in HHV-8associated MCD and idiopathic MCD, are believed to be driven by overproduction of interleukin-6 (IL-6).

• #2 Treatment guidelines (disease severity classification / disease activity index) |　Castleman disease, TAFRO, and related disease research group

  https://castleman.jp/en/introduction.html

  Castleman disease can present with various clinical symptoms of different degrees of severity. Frequent symptoms include low- to moderate-grade fever, general malaise, easy fatigability, weight loss, night sweats, and superficial lymphadenopathy. Some cases manifest with skin lesions (flat or slightly elevated brown or dark red eruptions, pemphigoids, xanthoma, atopic dermatitis, or hemangiomas), abdominal distension, edema, shortness of breath, dyspnea, and hemorrhagic tendency. Castleman disease can be associated with vascular events such as cerebral infarction, and peripheral neuropathy. […] In addition to lymphadenopathy, imaging examinations may reveal hepatosplenomegaly, ascites, pleural effusion, and pulmonary interstitial shadows. […] In some cases, renal dysfunction, interstitial pulmonary lesions, pulmonary hypertension, dilated cardiomyopathy, autoimmune thrombocytopenia, autoimmune hemolytic anemia, endocrinopathy (such as hypothyroidism), and/or AA-amyloidosis accompany the disease.

• #2 Idiopathic multicentric Castleman disease and associated autoimmune and autoinflammatory conditions: practical guidance for diagnosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10070070/

  The severity of peripheral neuropathy is less in CD patients with peripheral neuropathy but not concurrent POEMS, followed by POEMS-MCD and worst in classic POEMS without MCD. […] The coexistence of CD, inflammatory disorders and autoantibodies, in the same patient, including quite specific autoantibodies such as anti-Sjgren-syndrome-related antigen A (SSA) or anti-centromere, may be explained because they likely share some pathophysiology features.

• #2 Castleman Disease Clinical Presentation: History and Physical Examination

  https://emedicine.medscape.com/article/2219018-clinical

  HHV-8associated multicentric Castleman disease (HHV-8associated MCD) involves multicentric lymphadenopathy with characteristic „CD-like” lymph node histopathology and/or the following systemic manifestations, although some cases (10%) are asymptomatic: Flulike illness (fever, night sweats that soak the sheets, weight loss, loss of appetite, weakness, fatigue), Shortness of breath, cough, Nausea and vomiting, Numbness and weakness (neuropathy), Leg edema, Skin rashes, Hemangiomata, Pemphigus, Kaposi sarcoma. […] For idiopathic multicentric Castleman disease, according to the Castleman Disease Collaborative Network (CDCN), at least 2 of following must be present for it to be classified as severe: Eastern Cooperative Oncology Group (ECOG) performance status 2 or above, Stage IV kidney dysfunction (estimated glomerular filtration rate 30 mL/minute/1.73m2, creatinine 3 mg/dL), Anasarca and/or ascites, pleural effusion, or pericardial effusion, Hemoglobin 8 g/dL, Pulmonary involvement/interstitial pneumonitis with dyspnea.

• #2 How We Manage Idiopathic Multicentric Castleman Disease – Hematology & Oncology

  https://www.hematologyandoncology.net/archives/september-2022/how-we-manage-idiopathic-multicentric-castleman-disease/

  Patients demonstrate a range of signs and symptoms, including multicentric lymphadenopathy, an inflammatory syndrome, and fluid accumulation. […] In addition, multiorgan system dysfunction may develop as a consequence of the cytokine storm. […] The treatment of iMCD is complicated by a limited understanding of the etiology and pathogenesis. […] Disease is defined as severe if at least 2 of the following features are present: Eastern Cooperative Oncology Group (ECOG) performance status of at least 2, stage 4 renal dysfunction, extravascular fluid accumulation, hemoglobin level no higher than 8 g/dL, and evidence of pulmonary involvement. […] If evidence of disease progression develops in a patient with severe iMCD despite siltuximab, we recommend the prompt initiation of multiagent chemotherapy.

• #2 The French paediatric cohort of Castleman disease: a retrospective report of 23 patients | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-1345-5

  We identified 23 patients (12 girls) with a diagnosis of UCD (n=17) and MCD (n=6) between 1994 and 2018. The mean age at first symptoms was 11.47 4.23years for UCD and 8.3 3.4years for MCD. The mean diagnosis delay was 8.16 10.32months for UCD and 5.16 5.81years for MCD. In UCD, the initial symptoms were isolated lymph nodes (n=10) or lymph node associated with other symptoms (n=7); fever was present in 3 patients. Five patients with MCD presented fever. […] For patients with MCD, the mean age at the first symptoms was 8.33.4years (range 2.813). They presented fever (5/6; 83.3%), abdominal lymph nodes (5/6; 83.3%), failure to thrive (3/6; 50%), hepatomegaly and/or splenomegaly (3/6; 50%), arthralgia (2/6; 33.3%), abdominal pain (2/6; 33.3%), fatigue (2/6; 33.3%), facial oedema (1/6;16.7%), isolated lymphadenopathy (1/6; 16.7%), rash on the trunk (1/6; 16.7%), vascular hepatopathy with oesophageal varicose veins (1/6; 16.7%), diarrhoea (1/6; 16.7%) and cholestasis (1/6; 16.7%).

• #2 Idiopathic multicentric Castleman disease and associated autoimmune and autoinflammatory conditions: practical guidance for diagnosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10070070/

  Idiopathic multicentric Castleman disease (iMCD) is an infrequent and life-threatening disorder characterized by systemic inflammatory symptoms, generalized lymphadenopathy, polyclonal lymphocyte proliferation and organ dysfunction caused by a hyperinflammatory state. […] The main symptoms of iMCD are presented in Table 1. […] The inflammatory iMCD symptoms usually are intermittent, occurring in flares, whose precipitants are not clearly established. In more severe cases, once the inflammatory flare is established, it becomes self-sustained and may be life-threatening. […] All forms of MCD are characterized by a clinical presentation of systemic inflammatory symptoms, generalized lymphadenopathy (usually of relatively small volume), organ system dysfunction and laboratory abnormalities. The clinical spectrum of disease severity is very broad, ranging from minor symptoms to rapid, severe onset of symptoms that can result in life-threatening organ dysfunction.

• #2 Castleman Disease: Types, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17920-castleman-disease

  Castleman disease symptoms vary depending on the type. You likely wont have symptoms with unicentric Castleman disease. Enlarged lymph nodes may be the only sign. Sometimes, symptoms occur when an enlarged lymph node creates pressure on a nearby organ. […] MCD is more likely to cause noticeable symptoms. In addition to enlarged lymph nodes, signs and symptoms include: Fever, Fatigue (may be a sign of anemia), Night sweats, Nausea and vomiting, Unexplained weight loss, Swelling in your feet, ankles or abdomen, Enlarged spleen (splenomegaly) or liver (hepatomegaly), Numbness in your hands and feet (peripheral neuropathy). […] The prognosis (outlook) is good for most people with UCD. Removing the affected lymph node is often enough to cure it. With treatment, UCD doesnt usually affect life expectancy.

• #2 Signs & Symptoms – Castleman Disease Collaborative Network | CDCN

  https://cdcn.org/castleman-disease/signs-symptoms/

  In multicentric Castleman disease (MCD), enlarged lymph nodes appear in multiple regions of the body. Symptoms occur due to a systemic hyperinflammatory response and tend to be more severe than UCD symptoms. Flu-like symptoms (fever, night sweats, loss of appetite, nausea, and vomiting). Edema (swelling), ascites (fluid accumulation in the abdomen), and/or other symptoms of fluid accumulation. Unintentional weight loss. Weakness, fatigue. Enlarged liver or spleen. Kidney dysfunction. Peripheral neuropathy (numbness in the hands and feet). Cherry hemangioma skin rash.

• #2

  https://haematologica.org/article/view/haematol.2023.283603

  Following the iMCD diagnosis, 48% (n=49) of the cohort developed acute renal failure, 15.7% (n=16) developed chronic kidney disease, 13.7% (n=14) developed iron deficiency anemia, 10.7% (n=11) developed pneumonia, 6.9% (n=7) developed sepsis, and 6.9% (n=7) developed thrombotic microangiopathy. […] This study found that iMCD patients experienced a greater degree of hospitalization than the general population experiences in a given year, with this being primarily due to the increased rates of hospitalization among iMCD-TAFRO patients.

• #2 Multicentric Castleman’s Disease with Disseminated Kaposi’s Sarcoma: A Report of Prolonged Misdiagnosis

  https://clinmedjournals.org/articles/ijbrd/international-journal-of-blood-research-and-disorders-ijbrd-7-051.php?jid=ijbrd

  Castleman’s Disease is a rare and benign lymphoproliferative disease. […] The prognosis of untreated disease is poor and thus, early recognition and timely initiation of therapy are critical. […] A 54-year-old gentleman was diagnosed with acquired immunodeficiency syndrome (AIDS) when presenting with shingles in 2016. He was started on HAART but soon noted a very rapid increase in the size of his cervical, axillary and inguinal lymph nodes, along with profound constitutional symptoms. […] The patient was treated with liposomal doxorubin and rituximab without a clinical response. […] After completion of 6 planned cycles, a PET-CT confirmed a complete response and a repeat HHV-8 level was < 1000 copies/mL. Unfortunately, the patient then reported a rapidly progressive painful erythematous rash in both lower extremities. [...] Castleman's disease is a great mimicker of multiple illnesses due to its various manifestations. [...] This delay led to the aggressive widespread dissemination of the disease requiring a 5-drug regimen which is an uncommon approach in this disease.

• #3 Castleman disease | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/castleman-disease?lang=us

  Unicentric Castleman disease is usually asymptomatic and incidentally found on routine exams for other reasons. […] Multicentric Castleman disease has an exuberant clinical presentation due to the systemic inflammation, with symptoms such as fevers, night sweats, fatigue, and weight loss, and clinical signs of generalized lymphadenopathy, hepatosplenomegaly, and fluid retention. […] Hematological abnormalities such as anemia, elevated inflammatory markers, hypergammaglobulinemia, and hypoalbuminemia are commonly found in patients with multicentric Castleman disease. […] Multicentric Castleman disease may involve all of the above and is associated with a more complicated clinical course with systemic symptoms including fever and organomegaly. […] The prognosis is relatively poorer.

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