Multicentric, Hyaline Vascular Variant of Castleman’s Syndrome

Abstract

Castleman’s disease is a nonneoplastic adenopathy, also known as angiofollicular or giant lymphnode hyperplasia. Its multicentric form presents with wide spread lymphadenopathy and systemic symptoms. POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes) is observed in 11–30 % of the patients with Castleman’s disease (Dispenzieri et al., Blood 101:2496–2506, 2003). Tuberculosis and lymphoma are the common diagnosis in patients with clinical features of weight loss, lymphadenopathy, hepatosplenomegaly and lymphocyte predominant ascites. We had a similar presentation in a young male, who was a diabetic, with clinical features suggestive of probable tuberculous infiltration of abdomen and adrenals causing addisonian features, lymphocyte predominant ascites, hepatosplenomegaly and generalized lymphadenopathy. However, a pathological diagnosis of hyaline vascular variant of Castleman’s disease was obtained on lymphnode biopsy, which propelled us into doing a skeletal survey and bonemarrow biopsy. A final diagnosis of multicentric Castleman’s disease with reactive plasmacytosis was achieved. Patient had osteosclerotic lesion in the right femur with evidence of polyneuropathy, organomegaly, endocrinopathies and skin hyperpigmentation. He has improved on chemotherapy with melphan and dexamethasone. Hyaline vascular variant of Castleman’s disease rarely presents with systemic symptoms. Follow up is essential, as they can manifest with complications like POEMS and lymphoma.