Polyclonal lymphadenopathy presenting as plasma cell leukemia with reversible renal insufficiency

Acta Haematologica
L ResegottiD Testa

Abstract

A case is reported of an elderly patient with generalized lymphadenopathy who, at presentation, showed a blood and bone marrow picture suggestive of plasma cell leukemia, polyclonal hypergammaglobulinemia restricted almost exclusively to IgA, and severe renal insufficiency. Treatment with melphalan and prednisone produced a complete remission that lasted only 1 month. A second partial remission was obtained, but the patient eventually died of heart failure. The pathological picture of the lymph nodes and spleen was intermediate between that of angioimmunoblastic lymphadenopathy (AIL) and of the plasma cell type of giant lymph node hyperplasia (Castleman's disease). The hypothesis is presented that AIL, Castleman's disease and many other polyclonal lymphadenopathies recently described in immunodeficient or elderly patients, including the present case, represent a continuous spectrum of lymphoproliferative disorders due to abnormal responses to various stimuli.

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Castleman Disease

Castleman disease is a rare disorder that involves an overgrowth of cells in the lymph nodes. Unicentric Castleman disease affects one lymph node, usually in the chest or abdomen. Multicentric Castleman disease affects multiple lymph nodes, commonly located in the neck, collarbone, underarm and groin areas. Discover the latest research on Castleman disease here.