CLINICOPATHOLOGICAL SPECTRUM OF HEMOPHAGOCYTIC SYNDROME IN EPSTEIN-BARR VIRUS-ASSOCIATED PERIPHERAL T-CELL LYMPHOMA

Haemophagocytic syndrome (HS) is frequently observed in Epstein-Barr v irus-associated peripheral T-cell lymphoma (EBV-PTCL) and represents a major cause of death. In this communication we have further analysed the spectrum of HS in 12 patients with EBV-PTCL. The patients could be divided into three groups according to the time of onset of HS during the clinical course of PTCL. Group I patients (four cases) had HS as the initial clinical manifestation. All four patients were initially s uspected to have malignant histiocytosis (MH) but a MH-like PTCL was l ater diagnosed. Group II patients (six cases) developed HS at the time of lymphoma relapse. Four of them belonged to the angioinvasive type PTCL. Group III patients (two cases) developed HS at clinical remissio n; both were angioinvasive type PTCL. Nine patients had serological ev idence suggesting active EBV infection. The clinical course after the onset of HS was generally fulminant in each group with a median surviv al of only 44d despite combination chemotherapy and/or empirical thera py with high-dose immunoglobulin and corticosteroids in six patients. In conclusion, HS represents a severe complication of EBV-PTCL. Althou gh most patients develop HS at a time of active lymphoma, the syndrome may occur when the lymphoma is in remission. Because of the poor outc ome, early diagnosis and a new modality of treatment for HS associated with EBV-PTCL should be pursued in future.