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Peripheral T-cell lymphoma associated with hemophagocytic syndrome [see
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B Falini, S Pileri, I De Solas, MF Martelli, DY Mason, G Delsol, KC Gatter and M Fagioli
Institute of Internal Medicine, University of Perugia, Italy.
Nine patients with an acute disease characterized by high fever, loss of
weight, prominent hepatosplenomegaly, slight or no lymphadenopathy,
abnormal liver function tests, and profound pancytopenia are reported. In
all cases, the disease presented in the absence of any pre-existing disease
or immunosuppressive therapy. In seven of the nine patients, survival was
very short (mean = 7 weeks). Two patients are still alive: one had a
relapse 24 months after the initial diagnosis, while the other is in
complete remission. The main pathological feature was the infiltration of
the marrow, spleen and liver by neoplastic T cells, accompanied by an
exuberant hyperplasia of benign-looking, hemophagocytizing histiocytes. The
term "peripheral T-cell lymphoma with hemophagocytic syndrome" is proposed
for this condition. Retrospective analysis of stored paraffin material
(1949 to 1965) from the Radcliffe Infirmary files suggests that at least
some of the cases designated as "histiocytic medullary reticulosis" by
Scott and Robb- Smith were examples of the syndrome herein described.
Volume 75,
Issue 2,
pp. 434-444,
01/15/1990
Copyright © 1990 by The American Society of Hematology
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