Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Castleman's disease (CD) is a polyclonal lymphoproliferative disorder also known as giant nodular hyperplasia or angiofollicular lymph node hyperplasia. It is a rare disease often associated to human immunodeficiency virus (HIV) and human herpes virus 8 (HHV-8). Histopathological findings in Castleman's disease suggest an exaggerated response to antigenic stimuli seen in other diseases associated with immune activation, such as rheumatoid arthritis. An important aspect of its pathogenesis is the autonomous production of interleukin-6 (IL-6). In this disease, the clinical manifestations are associated to IL-6 serum levels, and surgical removal of the compromised lymph nodes or use of anti-IL-6 antibodies can slow down the symptoms. We describe a multicentric Castleman's disease in a young woman not associated to HHV-8 virus infection or immunosuppression. A short review of the literature follows the description of this clinical case.
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PMID:[Multicentric Castleman disease not associated with HHV-8 and HIV viruses]. 2562 30

Rituximab treatment may cause or exacerbate Kaposi's sarcoma (KS) in patients with human immunodeficiency virus (HIV)-associated multicentric Castleman's disease. Despite the widespread use of rituximab, rituximab-induced KS has not yet been reported in HIV-negative patients with diffuse large B cell lymphoma (DLBCL). We herein report a case of KS that developed after undergoing rituximab-containing chemotherapy in an HIV-negative patient with DLBCL. An 84-year-old man who received rituximab-containing chemotherapy for the treatment of DLBCL developed severe infection, and subsequently KS. Our observations indicate that serious infections under rituximab treatment may trigger KS. KS should therefore be considered when skin tumors appear in lymphoma patients receiving rituximab-containing chemotherapy.
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PMID:Rituximab-containing Chemotherapy (R-CHOP)-induced Kaposi's Sarcoma in an HIV-negative Patient with Diffuse Large B Cell Lymphoma. 2666 14

Thrombocytopenia (T), anasarca (A), myelofibrosis (F), renal dysfunction (R), and organomegaly (O) (TAFRO) syndrome is a variant of multicentric Castleman's disease. We describe here a 57-year-old man who presented with persistent fever, pleural effusion, and ascites. He was negative for human immunodeficiency virus and human herpes virus-8. A computed tomography scan showed an anterior mediastinal mass and small inguinal lymphadenopathy. Although a biopsy of the anterior mediastinum showed fatty tissue infiltrated with CD20 (+) and CD45RO (+) lymphocytes, a biopsy of the left inguinal lymph node revealed a hyaline vascular type of Castleman's disease. He subsequently developed severe thrombocytopenia and renal dysfunction. In addition, his bone marrow biopsy showed myelofibrosis. TAFRO syndrome was diagnosed based on the lymph node pathology and the characteristic manifestations of the syndrome. Tocilizumab and glucocorticoid therapy achieved complete remission and regression of the mediastinal mass. To our knowledge, this is the first report of TAFRO syndrome accompanied by an anterior mediastinal mass, which responded very well to therapy.
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PMID:An anterior mediastinal lesion in TAFRO syndrome showing complete remission after glucocorticoid and tocilizumab therapy. 2751 89

Multicentric Castleman's disease and Kaposi's sarcoma are more frequently observed in human immunodeficiency virus (HIV)-infected patients; however, 40-50% of the cases are HIV-negative. The present study reports the case of a 61-year-old man who presented with palpable masses in the axillary and right inguinal areas. The blood test results revealed increased serum erythrocyte sedimentation rate and C-reactive protein level, with negative serological markers, including for HIV. The patient was investigated using contrast-enhanced computed tomography (CT) and fluorine- 18fluorodeoxyglucose positron emission tomography (18F-FDG PET)/CT; the images revealed multiple enlarged and intensely hypermetabolic lymph nodes in the cervical, thoracic and abdominopelvic areas. Excisional biopsy and immunohistochemical analysis were performed, which confirmed the diagnosis of HIV-negative multicentric Castleman's disease coexisting with Kaposi's sarcoma. The patient received steroid therapy followed by chemotherapy. After 4 cycles of chemotherapy, the follow-up 18F-FDG PET/CT scan revealed nearly complete remission of the hypermetabolic malignant lesions of the neck, axilla and thoracoabdominal region.
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PMID:Human immunodeficiency virus-negative multicentric Castleman's disease coexistent with Kaposi's sarcoma on 18F-FDG PET/CT: A case report. 3068 Feb 14


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