HEMOPHAGOCYTOSIS IN BONE-MARROW ASPIRATE - A REVIEW OF THE CLINICAL COURSE OF 10 CASES

The clinical course of 10 cases where marrow aspirate showed features of haemophagocytosis was reviewed. Eight of these had a fulminant clin ical course characterised by high fever, constitutional symptoms, wast ing, hepatosplenomegaly with liver dysfunction, sometimes lymphadenopa thy, progressive pancytopenia and coagulopathy, like that described as malignant histiocytosis' in the past. The remaining 2 cases did not h ave this classical clinical syndrome. Among the former 8 cases, 4 of t hem 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 hav e fulminant clinical feature were found to have lymphoma of the diffus e large cell and Ki-l anaplastic type, respectively. A review of the l iterature 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 cla ssical haemophagocytic syndrome had a median survival of 24 days and a long-term survival of 37.5% at 28 months. Prompt initiation of chemot herapy is a life-saving measure and the only chance of achieving a lon g-term survival in patients with haemophagocytic syndrome if the under lying lymphoma can be diagnosed early.