The clinical course of 10 cases where marrow aspirate showed features of haemophagocytosis was reviewed. Eight of these had a fulminant clinical course characterised by high fever, constitutional symptoms, wasting, hepatosplenomegaly with liver dysfunction, sometimes lymphadenopathy, progressive pancytopenia and coagulopathy, like that described as ‘malignant histiocytosis’ in the past. The remaining 2 cases did not have this classical clinical syndrome. Among the former 8 cases, 4 of them had high-grade lymphoma, 3 of whom were confirmed to be peripheral T cell lymphoma. Three of the remaining 4 had suspicious lymphomatous infiltrate on marrow trephine. In every case an extensive search for viral etiology by serology was negative. The 2 cases which did not have fulminant clinical feature were found to have lymphoma of the diffuse large cell and Ki-1 anaplastic type, respectively. A review of the literature revealed that most cases with haemophagocytic syndrome have a fulminant clinical course and are peripheral T cell lymphoma, which generally has a poor prognosis. In our study, the 8 cases with the classical haemophagocytic syndrome had a median survival of 24 days and a long-term survival of 37.5% at 28 months. Prompt initiation of chemotherapy is a life-saving measure and the only chance of achieving a long-term survival in patients with haemophagocytic syndrome if the underlying lymphoma can be diagnosed early.

Keywords:
Haemophagocytosis, Haemophagocytic syndrome, High-grade lymphoma, Viral-associated haemophagocytic syndrome, Chemotherapy
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© 1995 S. Karger AG, Basel
1995
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