Lymphocyte-Depletion Hodgkin's Disease

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Abstract
Study of 13 cases of lymphocyte-depletion Hodgkin's disease disclosed a distinctive clinicopathological entity of rapidly fatal disease with fever, pancytopenia, lymphocytopenia and abnormalities of hepatic function, frequently without peripheral lymphadenopathy. In 10 patients the histologic type was that of diffuse fibrosis, and the diagnosis was frequently made by bone-marrow aspiration or laparotomy or at autopsy. The remaining three patients had the reticular type, with more obvious peripheral lymphadenopathy. The distribution of the process was predominantly subdiaphragmatic, with extensive involvement of liver, spleen, retroperitoneal lymph nodes and all bone-marrow sites, associated with marked marrow hypoplasia. Multiple bone-marrow examinations and liver biopsy in patients without peripheral lymphadenopathy may provide diagnostic material. The striking clinical presentation, distinctive organ distribution and marked cellular depletion of this entity differ from other types of Hodgkin's disease, and define it as a distinct clinicopathological syndrome.