Michael A. Baumann, MD; Joseph A. Libnoch, MD
JAMA. 1984;252(10):1340. doi:10.1001/jama.1984.03350100062034.
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Intense hemophagocytosis by bone marrow histiocytes was observed in a 66-year-old man who had fever, jaundice, hepatosplenomegaly, and pancytopenia. This constellation of findings was originally described by Scott and Robb-Smith1 in 1939 and was termed histiocytic medullary reticulosis because of the characteristic infiltration of the medullary region of the lymph nodes by histiocytes. Rappaport2 renamed the disease malignant histiocytosis in 1966 and emphasized the presence of cytologic atypia of the hemophagocytic histiocytes as a criterion for diagnosis. Malignant histiocytosis is now recognized as one of the rare malignancies of true monocytemacrophage origin and is generally regarded as having a poor prognosis, although some encouraging reports of improved survival following combination chemotherapy have been published in recent years.3

Hemophagocytosis has also been observed accompanying reactive histiocytic proliferation in response to systemic bacterial infections, including miliary tuberculosis, brucellosis, and typhoid.4 Recently, prominent hemophagocytosis was described in association with


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