Peripheral T-cell lymphoma associated with hemophagocytic syndrome

Brunangelo Falini, Stefano Pileri, Ignacio De Solas, Massimo F. Martelli, David Y. Mason, Georges Delsol, Kevin C. Gatter, Marta Fagioli

Research output: Contribution to journalArticlepeer-review

Abstract

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 benignlooking, 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.

Original languageEnglish
Pages (from-to)434-444
Number of pages11
JournalBlood
Volume75
Issue number2
Publication statusPublished - Jan 15 1990

ASJC Scopus subject areas

  • Hematology

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