Clinical Features Of Hivassociated Castlemans Disease

In general, MCD presents in the fourth or fifth decade of life but occurs earlier in people who are HIV positive. Patients often present with generalized malaise, night sweats, rigors, fever, anorexia, and weight loss. On examination, they have multiple lymphadenopa-thy, hepatosplenomegaly, ascites, edema, and effusions both pulmonary and pericardial. Laboratory investigations may reveal thrombocytopenia, anemia, hypoalbuminemia, and hypergammaglobulinemia. The systemic symptoms are attributed to IL-6 and can be severe enough to cause pancytopenia, organ failure, particularly respiratory and renal, as well as shock, requiring admission into intensive care units. HIV-infected patients with MCD have a greater preponderance for pulmonary complications. MCD is more likely to lead to neuropathic complications than does locally confined Castleman's disease. Patients can develop polyneuropathies, leptomeningeal and CNS infiltration, as well as myasthenia gravis.114 The polyneuropathy is a chronic inflammatory demyeli-nating neuropathy and may be present as part of the rare POEMS syndrome (Crow-Fukase disease). POEMS syndrome consists of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. Patients are diagnosed with POEMS syndrome if they have two of these clinical features as well as plasma cell dyscrasia. Not only is MCD itself potentially fatal due to organ failure, but it is also associated with a 15-fold increased incidence of NHL. The majority of these lymphomas are plasmablastic and are thought to arise from expansion of plasmablastic microlymphomas seen in MCD lesions.112113

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