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Castleman Disease

Castleman disease, also known as angiofollicular lymph node hyperplasia and giant lymph node hyperplasia, represents a group of non-neoplastic lymphoproliferative disorders sometimes associated with viral (human herpesvirus 8 and human immunodeficiency virus) infections. This condition has two subtypes, unicentric and multicentric, that dictate prognosis.


Presentation

Castleman disease patients may be asymptomatic, especially if suffering from the unicentric (localized) type. When the disease is not clinically silent, patients complain about the presence of slow growing localized mass that may cause neighboring structures compression, depending on its location and size. The multicentric subtype is characterized by multiple lymphadenopathy. Both forms may be accompanied by systemic manifestations of the disease, consisting of fever, fatigue, abundant nocturnal sweating, inappetence, nausea, vomiting, cough, and dyspnea, as well as hepatomegaly, splenomegaly, peripheral edema, ascites, pleural effusions, and cutaneous signs, such as hemangiomata, rashes, or pemphigus [1] [2]. Symptoms may follow a bimodal pattern, with remissions and relapses. In patients with human immunodeficiency virus infection, Kaposi sarcoma or primary effusion lymphoma may also be noticed [3].

Amyloidosis, a complication of this illness, causes symptoms that sometimes overlap with those of multicentric Castleman disease, making the diagnosis more difficult. Castleman disease treatment improves amyloidosis manifestations, as well. If the treatment is delayed, multicentric Castleman disease associated with human herpesvirus 8 is fatal within two years by multiorgan failure, infections, or immunoblastic lymphoma development [4] [5].

Idiopathic multiple Castleman disease is more frequent in men in their fourth or fifth decade of life [6]. Different ethnic groups have various clinical traits. For example, Asian individuals frequently have interstitial pneumonitis and large violaceous skin lesions [7], while Polynesian individuals usually have a mild form of the disease [8].

Splenomegaly
  • Older age ( 40 years), splenomegaly, and hypoalbuminemia were risk factors for poorer MCD prognosis.[ncbi.nlm.nih.gov]
  • The disease is characterized by fever, weight loss, anemia, polyclonal hyperglobulinemia, splenomegaly, thrombocytosis and peripheral lymphadenopathy.[ncbi.nlm.nih.gov]
  • Facing a patient with fever, peripheral lymphadenopathy, splenomegaly, and the biochemical and immunological picture depicted above, the physician may find it difficult to pinpoint the exact diagnosis.[symptoma.com]
  • Nevertheless, splenomegaly persisted in the first patient, and a secondary hepatic node appeared in the second patient. The side effects, essentially sustained thrombocytopenia, were mild in both cases.[ncbi.nlm.nih.gov]
  • Patients with iMCD and HHV-8 MCD demonstrated similar characteristics, including fever, splenomegaly, cytopenia and inflammatory symptoms. However, the disease was more aggressive in HHV-8 MCD, particularly in HIV-infected patients.[ncbi.nlm.nih.gov]
Generalized Lymphadenopathy
  • Case Report: We present a case of a 63-year-old woman with multicentric CD who was admitted with weight loss, night sweat, fever, and generalized lymphadenopathy.[karger.com]
  • HHV-8–Positive MCD HHV-8–positive MCD presents with generalized lymphadenopathy and constitutional symptoms, and can progress to multi-organ failure leading to death.[hematology.org]
  • Physical examination will mainly demonstrate presence of either localized or generalized lymphadenopathy.[cancertherapyadvisor.com]
  • In contrast, the multicentric form is found in older adults and is usually associated with the plasma cell variant, a systemic disease with generalized lymphadenopathy, hepatosplenomegaly, fever, and night sweats; in addition, it is frequently associated[revespcardiol.org]
  • Diagnosis A high index of suspicion is required in patients presenting with a compatible clinical picture, such as unifocal or generalized lymphadenopathy, splenomegaly, and/or unexplained constitutional symptoms (Chan K et al., 2016).[atlasgeneticsoncology.org]
Cervical Lymphadenopathy
  • In this case report, we discuss the case of a 7-year-old boy with multicentric plasma cell variant of CD, who presented with cervical lymphadenopathies, autoimmune hemolytic anemia, bone marrow insufficiency, pulmonary, renal, hepatic, and gastrointestinal[ncbi.nlm.nih.gov]
  • However, in case 1, subsequent flow cytometric analysis at the time of recurrent cervical lymphadenopathy detected a monoclonal B-cell population with CD10 coexpression.[academic.oup.com]
  • Symptoms are usually related to the area of lymph node enlargement which includes, in a classic case, a large mediastinal mass with pulmonary symptoms or palpable cervical lymphadenopathy.[cancertherapyadvisor.com]
Cough
  • Both patients had pulmonary symptoms and signs, including cough, dyspnoea, hypoxaemia and ventilatory dysfunction; however, they had different physiological manifestations of their pulmonary abnormalities.[ncbi.nlm.nih.gov]
  • CD evolving into intrabronchial, extramedullary plasmacytoma.Intrabronchial mass with consequential obstruction of the left main bronchus, left lung atelectasis and mediastinal lymphadenomegaly was detected by chest CT in our patient suffering from cough[ncbi.nlm.nih.gov]
  • Coughing (72.7%), fever (68.2%), and dyspnea (59.1%) were the common clinical manifestations.[ncbi.nlm.nih.gov]
  • Both forms may be accompanied by systemic manifestations of the disease, consisting of fever, fatigue, abundant nocturnal sweating, inappetence, nausea, vomiting, cough, and dyspnea, as well as hepatomegaly, splenomegaly, peripheral edema, ascites, pleural[symptoma.com]
  • Symptoms and signs of MCD include fever, night sweats, loss of appetite, nausea/vomiting, weight loss, cough, shortness of breath, weakness or fatigue from anemia, enlarged lymph nodes (around the neck, collarbone, underarm, and groin area), enlarged[secure.ssa.gov]
Dyspnea
  • A 36-year-old woman suffering from oral ulcerations, skin rash, and dyspnea was suspected of having paraneoplastic pemphigus.[ncbi.nlm.nih.gov]
  • We present the case of a 76 year old female patient admitted in the Department of Cardiology for physical asthenia, profuse sweating and dyspnea with orthopnea for about one month.[ncbi.nlm.nih.gov]
  • Coughing (72.7%), fever (68.2%), and dyspnea (59.1%) were the common clinical manifestations.[ncbi.nlm.nih.gov]
  • Both forms may be accompanied by systemic manifestations of the disease, consisting of fever, fatigue, abundant nocturnal sweating, inappetence, nausea, vomiting, cough, and dyspnea, as well as hepatomegaly, splenomegaly, peripheral edema, ascites, pleural[symptoma.com]
  • We present the case of a 66-year-old man with no known cardiovascular risk factors who complained of dyspnea upon moderate exertion, together with a weight loss of 4 to 5 kg over a 6-month period.[revespcardiol.org]
Orthopnea
  • We present the case of a 76 year old female patient admitted in the Department of Cardiology for physical asthenia, profuse sweating and dyspnea with orthopnea for about one month.[ncbi.nlm.nih.gov]
Fever
  • A 46-year-old male patient presented with fever for over 5 months. An FDG PET/CT was acquired to evaluate the source of the fever.[ncbi.nlm.nih.gov]
  • The disease is characterized by fever, weight loss, anemia, polyclonal hyperglobulinemia, splenomegaly, thrombocytosis and peripheral lymphadenopathy.[ncbi.nlm.nih.gov]
  • Facing a patient with fever, peripheral lymphadenopathy, splenomegaly, and the biochemical and immunological picture depicted above, the physician may find it difficult to pinpoint the exact diagnosis.[symptoma.com]
  • He was found to have a remittent fever and a lower mid-abdominal tumor. Blood test revealed microcytic hypochromic anemia, thrombocytosis, polyclonal hypergammaglobulinemia, hyperfibrinogenemia, and elevated erythrocyte sedimentation rate.[ncbi.nlm.nih.gov]
  • We describe herein the case of an adolescent girl with anemia non-responsive to oral iron, associated with low-grade fever, diminished appetite and fatigue. A palpable mass below the xiphoid was noted.[ncbi.nlm.nih.gov]
Weight Loss
  • The disease is characterized by fever, weight loss, anemia, polyclonal hyperglobulinemia, splenomegaly, thrombocytosis and peripheral lymphadenopathy.[ncbi.nlm.nih.gov]
  • Also call your doctor if you experience a persistent feeling of fullness in your chest or abdomen, fever, fatigue, or unexplained weight loss. Causes It's not clear what causes Castleman disease.[mayoclinic.org]
  • Symptoms include: High fever Anemia Weight loss Loss of appetite Low white blood cell counts Castleman’s disease can also be defined as hyaline vascular or plasma cell type. This is based on the appearance of the lymph node tissue.[uamshealth.com]
  • The patient reported an episode of self-limiting haemoptysis one month previously, weight loss that she related with anxiety, occasional retrosternal oppression accompanied by paraesthesia in the left arm and episodes of bronchospasm.[archbronconeumol.org]
  • Case Report: We present a case of a 63-year-old woman with multicentric CD who was admitted with weight loss, night sweat, fever, and generalized lymphadenopathy.[karger.com]
Anemia
  • Chronic, iron-refractory, microcytic anemia can be a diagnostic and therapeutic challenge.[ncbi.nlm.nih.gov]
  • Laboratory findings were consistent with anemia of inflammation. Direct antiglobulin test was positive without any other evidence of autoimmune anemia. Other autoantibodies, such as anti-thyroid and anti-nuclear antibodies, were also positive.[ncbi.nlm.nih.gov]
  • The disease is characterized by fever, weight loss, anemia, polyclonal hyperglobulinemia, splenomegaly, thrombocytosis and peripheral lymphadenopathy.[ncbi.nlm.nih.gov]
  • In this case report, we discuss the case of a 7-year-old boy with multicentric plasma cell variant of CD, who presented with cervical lymphadenopathies, autoimmune hemolytic anemia, bone marrow insufficiency, pulmonary, renal, hepatic, and gastrointestinal[ncbi.nlm.nih.gov]
  • Blood test revealed microcytic hypochromic anemia, thrombocytosis, polyclonal hypergammaglobulinemia, hyperfibrinogenemia, and elevated erythrocyte sedimentation rate. The serum IL-6 and C-reactive protein levels were increased.[ncbi.nlm.nih.gov]
Lymphadenopathy
  • The remaining 6 referral cases showed morphologic features consistent with reactive lymphadenopathy.[ncbi.nlm.nih.gov]
  • Castleman disease (CD) is a neoplasm that presents with single or multiple lymphadenopathy. The disease is characterized by fever, weight loss, anemia, polyclonal hyperglobulinemia, splenomegaly, thrombocytosis and peripheral lymphadenopathy.[ncbi.nlm.nih.gov]
  • Castleman disease is a rare lymphoproliferative disorder presenting with localized or disseminated lymphadenopathy and systemic manifestations.[ncbi.nlm.nih.gov]
  • As treatment for Castleman disease, she was administered a combination of rituximab and etoposide, which led to a reduction in lymphadenopathy.[ncbi.nlm.nih.gov]
  • In this case report, we discuss the case of a 7-year-old boy with multicentric plasma cell variant of CD, who presented with cervical lymphadenopathies, autoimmune hemolytic anemia, bone marrow insufficiency, pulmonary, renal, hepatic, and gastrointestinal[ncbi.nlm.nih.gov]
Fatigue
  • We describe herein the case of an adolescent girl with anemia non-responsive to oral iron, associated with low-grade fever, diminished appetite and fatigue. A palpable mass below the xiphoid was noted.[ncbi.nlm.nih.gov]
  • The patient was initially treated with antiretroviral therapy with a combination of elvitegravir, cobicistat, emtricitabine and tenofovir that improved her fatigue and malaise.[ncbi.nlm.nih.gov]
  • The lymphoma patients presented significantly more often with fatigue and fever (p   0.023 and p   0.016 respectively) than did the CD subjects.[ncbi.nlm.nih.gov]
  • Grade- 3 adverse events (AEs) reported in 1 patient included hypertension (n 3) and nausea, cellulitis, and fatigue (n 2 each).[ncbi.nlm.nih.gov]
  • Also call your doctor if you experience a persistent feeling of fullness in your chest or abdomen, fever, fatigue, or unexplained weight loss. Causes It's not clear what causes Castleman disease.[mayoclinic.org]
Nausea
  • Grade- 3 adverse events (AEs) reported in 1 patient included hypertension (n 3) and nausea, cellulitis, and fatigue (n 2 each).[ncbi.nlm.nih.gov]
  • Both forms may be accompanied by systemic manifestations of the disease, consisting of fever, fatigue, abundant nocturnal sweating, inappetence, nausea, vomiting, cough, and dyspnea, as well as hepatomegaly, splenomegaly, peripheral edema, ascites, pleural[symptoma.com]
  • Some known complementary treatment methods include: Medication to reduce stress Acupuncture to decrease pain Peppermint tea for nausea Some of these methods have been helpful, while the others have not been so effective.[dovemed.com]
  • Symptoms and signs of MCD include fever, night sweats, loss of appetite, nausea/vomiting, weight loss, cough, shortness of breath, weakness or fatigue from anemia, enlarged lymph nodes (around the neck, collarbone, underarm, and groin area), enlarged[secure.ssa.gov]
  • 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[mayoclinic.org]
Vomiting
  • Both forms may be accompanied by systemic manifestations of the disease, consisting of fever, fatigue, abundant nocturnal sweating, inappetence, nausea, vomiting, cough, and dyspnea, as well as hepatomegaly, splenomegaly, peripheral edema, ascites, pleural[symptoma.com]
  • Symptoms and signs of MCD include fever, night sweats, loss of appetite, nausea/vomiting, weight loss, cough, shortness of breath, weakness or fatigue from anemia, enlarged lymph nodes (around the neck, collarbone, underarm, and groin area), enlarged[secure.ssa.gov]
  • Symptoms for MCD can include Fever and night sweats Nausea and vomiting Loss of appetite Weight loss A buildup of fluid in your belly Bruising and easy bleeding Cherry-like spots on your skin Numbness in your hands and feet Anemia -- having too few red[webmd.com]
  • Signs and symptoms of Castleman disease occur most often with the multicentric form and can include fever , night sweats , loss of appetite, nausea , vomiting , and fatigue .[medicinenet.com]
  • Skin rashes, weakness, nausea and vomiting, and a loss of appetite, are common side effects with radiation therapy Corticosteroid drugs: These drugs inhibit the immune system, thus decreasing the abnormal proliferation of lymphocytes.[dovemed.com]
Loss of Appetite
  • Symptoms include: High fever Anemia Weight loss Loss of appetite Low white blood cell counts Castleman’s disease can also be defined as hyaline vascular or plasma cell type. This is based on the appearance of the lymph node tissue.[uamshealth.com]
  • Symptoms and signs of MCD include fever, night sweats, loss of appetite, nausea/vomiting, weight loss, cough, shortness of breath, weakness or fatigue from anemia, enlarged lymph nodes (around the neck, collarbone, underarm, and groin area), enlarged[secure.ssa.gov]
  • Symptoms for MCD can include Fever and night sweats Nausea and vomiting Loss of appetite Weight loss A buildup of fluid in your belly Bruising and easy bleeding Cherry-like spots on your skin Numbness in your hands and feet Anemia -- having too few red[webmd.com]
  • Signs and symptoms of Castleman disease occur most often with the multicentric form and can include fever , night sweats , loss of appetite, nausea , vomiting , and fatigue .[medicinenet.com]
  • Skin rashes, weakness, nausea and vomiting, and a loss of appetite, are common side effects with radiation therapy Corticosteroid drugs: These drugs inhibit the immune system, thus decreasing the abnormal proliferation of lymphocytes.[dovemed.com]
Epigastric Pain
  • A 48-year-old woman presented with epigastric pain. CT scan showed a well-encapsulated mass on the ventral border of the pancreas. Endosonography with fine needle aspiration biopsy was performed.[ncbi.nlm.nih.gov]
Hepatosplenomegaly
  • 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 generalised lymphadenopathy, hepatosplenomegaly, and fluid[radiopaedia.org]
  • […] or discomfort causing difficulty with eating or swallowing Presence of a lump in the groin , neck , or armpit Unintentional loss of weight In some cases fever and weakness Easy fatigue Appetite loss or extremely poor appetite Lymph node enlargement Hepatosplenomegaly[epainassist.com]
  • Plasmacytic or plasmablastic Yes Positive for HHV-8 by QPCR May be positive for HIV Rituximab etoposide Optional valganciclovir maintenance Multicentric HHV-8-Negative (Idiopathic) Generalized hepatosplenomegaly Mostly plasmacytic, but can be hyaline[hematology.org]
Hepatomegaly
  • Examination revealed pallor; generalized edema; axillary, cervical, and inguinal lymphadenopathy; hypertension; and hepatomegaly.[ncbi.nlm.nih.gov]
  • MCD were more symptomatic (50% vs 96.9%, P   0.001) and sicker than UCD, including more fever, hepatomegaly and/or splenomegaly and hypoalbuminemia.[ncbi.nlm.nih.gov]
  • Both forms may be accompanied by systemic manifestations of the disease, consisting of fever, fatigue, abundant nocturnal sweating, inappetence, nausea, vomiting, cough, and dyspnea, as well as hepatomegaly, splenomegaly, peripheral edema, ascites, pleural[symptoma.com]
  • […] concentric sheets of plasma cells surround germinal centers 1 Histologic characteristics: hyalinization and hypervascularity in germinal centers 1 Associated with an array of systemic symptoms, including 3,4 : Fever, night sweats Anorexia/weight loss Hepatomegaly[castlemansconnect.com]
  • The main systemic symptoms of the disease are fever, fatigue, splenomegaly, and hepatomegaly, skin rash, and severe growth retardation.[mct.aacrjournals.org]
Night Sweats
  • Herein we present the case of a 49-year-old female with a 3-year history of progressively worsening lymphadenopathy associated with fevers, chills and night sweats. Laboratory studies showed anemia and mildly elevated sedimentation rate.[ncbi.nlm.nih.gov]
  • The recurrent high fevers, night sweats, sky-high inflammatory markers—like C-reactive protein or erythrocyte sedimentation rate—are attributable to IL-6 overexpression.[targetedonc.com]
  • Case Report: We present a case of a 63-year-old woman with multicentric CD who was admitted with weight loss, night sweat, fever, and generalized lymphadenopathy.[karger.com]
  • Symptoms and signs of MCD include fever, night sweats, loss of appetite, nausea/vomiting, weight loss, cough, shortness of breath, weakness or fatigue from anemia, enlarged lymph nodes (around the neck, collarbone, underarm, and groin area), enlarged[secure.ssa.gov]
  • sweats Anorexia/weight loss Hepatomegaly/splenomegaly Pericardial/pleural effusions Localized symptoms associated with compression in affected area—systemic symptoms are uncommon 3 CD is not considered a malignant disease; however, affected patients[castlemansconnect.com]

Workup

Blood workup in Castleman disease should include a complete blood count which will highlight the presence of hemolytic anemia, most often of a mild or moderate level, and that of thrombocytosis, but thrombocytopenia is also possible. High platelet numbers, together with an increase of acute phase reactants such as the fibrinogen, erythrocyte sedimentation rate, and C-reactive protein are caused by high circulating levels of interleukin 6 [2] [9]. Vascular endothelial growth factor and other cytokines were also found to be elevated [10]. Other characteristic findings are the presence of hypoalbuminemia and hypergammaglobulinemia. Hepatitis B, human immunodeficiency virus, and human herpesvirus 8 serologies are parts of the workup.

Facing a patient with fever, peripheral lymphadenopathy, splenomegaly, and the biochemical and immunological picture depicted above, the physician may find it difficult to pinpoint the exact diagnosis. In this case, a computed tomography guided lymph node biopsy should be performed. The material obtained should be examined from a histopathological point of view, given that the hyaline vascular type usually has a favorable outcome, as opposed to the plasmablastic type. The plasma cell type is likely to have multiple locations, whereas the mixed subtype was seldom described. Genetic studies, as well as fluorescent in situ hybridization, should be performed in order to exclude lymphoma or other clonal disorders.

Imaging studies are used to determine the extent of the disease. A thoracic radiography can show a wide mediastinum or pleural effusions. More detailed information is offered by computed tomography or positron emission tomography studies of the thorax, abdomen, pelvis, and neck [11].

Microcytic Anemia
  • Chronic, iron-refractory, microcytic anemia can be a diagnostic and therapeutic challenge.[ncbi.nlm.nih.gov]
  • anemia For disease progression of multicentric disease, some clinicians follow an IL-6 level and/or a HHV-8 viral load E.[clinicaladvisor.com]
  • Laboratory findings include hypochromic microcytic anemia, increased erythrocyte sedimentation rate and polyclonal hypergammaglobulinemia.[eurorad.org]
Human Herpesvirus 8
  • Hepatitis B, human immunodeficiency virus, and human herpesvirus 8 serologies are parts of the workup.[symptoma.com]
  • Castleman disease is a rare entity, including unicentric Castleman disease (UCD), human herpesvirus-8 plus Castleman disease (HHV-8 MCD), and idiopathic multicentric Castleman disease (iMCD).[ncbi.nlm.nih.gov]
  • The cause and pathogenesis of the four subtypes of CD (unicentric CD; human herpesvirus-8-associated multicentric CD; polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes [POEMS]-associated multicentric[ncbi.nlm.nih.gov]
  • In MCD caused by Kaposi sarcoma-associated herpesvirus/human herpesvirus-8 (KSHV/HHV8), the cytokine over activity is caused by viral products, which can also lead to atypical lymphoproliferations and potential progression to lymphoma.[ncbi.nlm.nih.gov]
  • Multicentric Castleman disease (MCD) is a lymphoproliferative disorder caused by human herpesvirus 8 (HHV8) infection HIV associated MCD (HIV-MCD) presents with various clinical symptoms.[ncbi.nlm.nih.gov]

Treatment

  • All MCD patients in this extension study have received siltuximab for a prolonged duration (up to 7 years) without evidence of cumulative toxicity or treatment discontinuations and with few serious infections.[ncbi.nlm.nih.gov]
  • Our report, in addition to data presented in the literature, suggests that tocilizumab could be an initial treatment option in patients with HIV-MCD.[ncbi.nlm.nih.gov]
  • T-lymphoblastic lymphoma is an aggressive neoplasm requiring prompt clinical treatment.[ncbi.nlm.nih.gov]
  • Siltuximab (anti-IL-6 antibody) is approved for treatment of this disease. To our knowledge, genomic sequencing of CD has not been reported.[ncbi.nlm.nih.gov]
  • You go to hospital 3 times in the first cycle of treatment and once in each treatment cycle after that. You have an ECG in cycle 4 and then every 6 months for as long as you have treatment.[cancerresearchuk.org]

Prognosis

  • Multicentric CD is classically associated with systemic symptoms and poorer prognosis.[ncbi.nlm.nih.gov]
  • Older age ( 40 years), splenomegaly, and hypoalbuminemia were risk factors for poorer MCD prognosis.[ncbi.nlm.nih.gov]
  • Prognosis: For localized Castleman's, the prognosis is actually quite good. However, there may be a recurrence of the condition. For multicentric Castleman's, the prognosis is not so good.[lymphedemapeople.com]
  • Most of MCD cases were arranged with chemotherapy and their prognosis were worse than UCD's.[ncbi.nlm.nih.gov]
  • This condition has two subtypes, unicentric and multicentric, that dictate prognosis. Castleman disease patients may be asymptomatic, especially if suffering from the unicentric (localized) type.[symptoma.com]

Etiology

  • Castleman disease is a rare lymphoproliferative disorder of unknown etiology. The localized form, which usually presents as a slow-growing mass, is most commonly located in the mediastinum.[ncbi.nlm.nih.gov]
  • Castleman disease is a rare lymphoproliferative disorder of unknown etiology that most commonly presents as a mediastinal nodal mass or, in the extranodal form of the disease, a mass located in the mediastinum or retroperitoneum.[ncbi.nlm.nih.gov]
  • Castleman disease (CD) is uncommon non-clonal lymphoproliferative disorder with unknown etiology. No standard therapy is recommended for relapsed/refractory CD patients, thus requiring development of novel experimental approaches.[ncbi.nlm.nih.gov]
  • While a cohort of multicentric Castleman disease (MCD) cases are caused by Human Herpes Virus-8 (HHV-8), the etiology of HHV-8 negative, idiopathic MCD (iMCD), remains unknown.[ncbi.nlm.nih.gov]
  • Even though the exact mechanism of pathogenesis is unknown, treatment is directed toward possible etiologies such as interleukin-6, cluster of differentiation 20, and viral agents.[ncbi.nlm.nih.gov]

Epidemiology

  • Improved definitions should improve understanding of the epidemiology of Castleman disease and its subtypes.[ncbi.nlm.nih.gov]
  • Epidemiology The epidemiology of CD is difficult to characterize accurately due to its rarity and clinical heterogeneity.[atlasgeneticsoncology.org]
  • Epidemiology Castleman’s disease (CD), from being rare, is now more commonly encountered. In the last three decades since the discovery of HIV, the incidence of MCD has progressively increased over time.[intechopen.com]
  • Epidemiology Castleman disease is rare.[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • The purpose of this article is to review the clinical, immunologic, and pathologic findings associated with both unicentric Castleman disease and MCD and discuss how the imaging findings correlate with the pathophysiology of the disease.[ncbi.nlm.nih.gov]
  • Pathophysiology The pathophysiology of Castleman disease (CD) varies between subtypes. Unicentric CD (UCD) pathophysiology is poorly understood.[emedicine.medscape.com]
  • Evidence that the pathophysiology of CD is fueled by excessive interleukin-6 (IL-6) has led to considerable interest in therapeutic targeting of this cytokine.[dovepress.com]
  • […] antibodies to interleukin-6 and its receptor, allow for more targeted disease-specific intervention that promises improved response rates and more durable disease control; however, further work is required to fill knowledge gaps in terms of underlying pathophysiology[dovepress.com]

Prevention

  • Right hemihepatectomy and hilar lymph node dissection was performed and preventative transcatheter arterial chemoembolization therapy was given after the surgery. The case had multiple systemic metastasis due to tumor progression.[ncbi.nlm.nih.gov]
  • CATIE ensures that these resources, developed to help prevent the transmission of HIV, hepatitis C and other infections, are written and reviewed by health experts for content accuracy.[catie.ca]
  • To prevent the need for a disability appeal , you should consider retaining the services of a qualified Social Security Disability attorney or advocate.[disabilitybenefitscenter.org]

References

Article

  1. Oksenhendler E, Duarte M, Soulier J, et al. Multicentric Castleman's disease in HIV infection: a clinical and pathological study of 20 patients. AIDS. 1996;10(1):61–67.
  2. van Rhee F, Fayad L, Voorhees P, et al. Siltuximab, a novel anti-interleukin-6 monoclonal antibody, for Castleman’s disease. J Clin Oncol. 2010;28: 3701–3708.
  3. Chadburn A, Hyjek EM, Tam W, et al. Immunophenotypic analysis of the Kaposi sarcoma herpesvirus (KSHV; HHV-8)-infected B cells in HIV+ multicentric Castleman disease (MCD). Histopathology. 2008;53(5):513–524.
  4. Dispenzieri A, Armitage JO, Loe MJ, et al. The clinical spectrum of Castleman’s disease. Am J Hematol. 2012;87(11):997-1002.
  5. Mylona EE, Baraboutis IG, Lekakis LJ, et al. Multicentric Castleman's disease in HIV infection: a systematic review of the literature. AIDS Rev. 2008;10(1):25–35.
  6. Nishimoto N, Kanakura Y, Aozasa K, et al. Humanized anti-interleukin-6 receptor antibody treatment of multicentric Castleman disease. Blood 2005;106(8):2627-2632.
  7. Ahmed B, Tschen JA, Cohen PR, et al. Cutaneous Castleman’s disease responds to anti interleukin-6 treatment. Mol Cancer Ther 2007;6(9):2386-2390.
  8. Zhai S. Polynesian variant of idiopathic multicentric Castleman disease [abstract]. Blood. 2013;122(21). Abstract 5127.
  9. Kishimoto T. IL-6: from its discovery to clinical applications. Intern Immunol. 2010;22:347–352
  10. Nakahara H, Song J, Sugimoto M, et al. Anti-interleukin-6 receptor antibody therapy reduces vascular endothelial growth factor production in rheumatoid arthritis. Arthr Rheumat. 2003; 48:1521–1529.
  11. Barker R, Kazmi F, Stebbing J, et al. FDG-PET/CT imaging in the management of HIV-associated multicentric Castleman's disease. Eur J Nucl Med Mol Imaging. 2009;36(4):648-652.

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Last updated: 2018-06-22 07:06