Systemic Weber-Christian Disease and Thrombocythemia Terminating in Reticulum-cell Sarcoma

Abstract
A 62-year-old Caucasian woman with idiopathic thrombocythemia treated with P-32 and chlorambucil developed Weber-Christian disease and reticulum-cell sarcoma 9 years and 6 months, respectively, before death. She also had a long history of hypogammaglobulinemia, rheumatoid arthritis, and chronic obstructive pulmonary disease. Necropsy revealed healed subcutaneous and active visceral panniculitis, a diffuse necrotizing angiitis, and extramedullary hematopoiesis. A review of the pathologic findings in Weber-Christian disease is presented. It is suggested that the development of the reticulum-cell sarcoma is related to thrombocythemia, treatment with P-32, prolonged chlorambucil therapy, and hypogammaglobulinemia.

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