Treatment Of Hivassociated Castlemans Disease

There are no definitive gold standard treatments for MCD. No randomized trials have been conducted on account of the infrequency of the diagnosis, and often only case reports have appeared in the literature. Although surgery is the mainstay of treatment for localized Castleman's disease, with complete removal of the mediastinal lesions being curative, it has a limited role in MCD. Splenectomy, in addition to establishing the histologic diagnosis, may have a therapeutic benefit as a debulking procedure, as some of the hematologic sequelae such as thrombocytopenia and anemia may in part be due to splenomegaly. Following splenectomy, there is often resolution of the constitutive symptoms but this may be short lived, and some form of maintenance therapy is needed to prevent relapse.115

For immunocompetent patients, the chemotherapy regimens for MCD are based on lymphoma schedules such as CHOP. However, in the pre-HAART era, these schedules were associated with marked toxicity in HIV-positive patients, and as a consequence, other schedules were developed. In the largest published study from Paris of 20 patients, there was a partial response in 9/9 patients with single agent vinblastine; however, only 4 patients remained stable with maintenance therapy (4-6 mg/2 weeks). Four patients received upfront ABV (adriamycin, bleomycin, and vincristine), three achieving a partial remission. Intermittent treatment with cyclophosphamide

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