Serous fluid cytology as a means of detecting hemophagocytosis in Epstein-Barr virus-induced autoimmune hemolytic anemia

The case of, a 22-yr-old incite who after a brief febrile episode developed autoimmune hemolytic anemia and right pulmonary infiltrate with pleural ef fusion is presented. Cytologic examination on of the pleural fluid revealed lymphocytosis and hemophagocytosis, primarily of red blood cells (RBCs) by mature histiocytes. There was accompanying splenomegaly, laboratory eviden ce of hepatic dysfunction, and retroperitoneal lymphadenopathy. Besides pro found reduction of red blood cells in the peripheral blood, there was reduc tion of lymphocytes and platelets. As a neoplastic process was rated out by bone marrow and pleural biopsies, the disease was considered to be virus-i nduced and was halted and progressively regressed with early institution of vigorous antiflammatory therapy with adrenocortical steroids. Upon reviewi ng the case, examination of the bone marrow biopsy disclosed limited hemoph agocytosis of RBCs and lymphocytes by histiocytes and considerable viral cy topathic effect on hematopoietic cells (red and white cell precursors and m egakaryocytes), which by appropriate immunolabelling was identified as indu ced by Epstein-Barr virus. A virus-related acquired hemophagocytic syndrome in its early stages was probably present, yet an undesirable clinical outc ome was averted by early institution of vigorous steroid therapy. The need to recognize early hemophagocytic changes in cytologic specimens for early institution of appropriate therapy is emphasized. The possibility of erythr ophagocytosis, also manifested during the course of an autoimmune hemolytic process and unrelated to hemophagocytic syndrome, is discussed. (C) 2001 W iley-Liss, Inc.