Clinicopathological spectrum of haemophagocytic syndrome in Epstein-Barr virus-associated peripheral T-cell lymphoma.

Haemophagocytic syndrome (HS) is frequently observed in Epstein-Barr virus-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 suspected to have malignant histiocytosis (MH) but a MH-like PTCL was later 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 remission; both were angioinvasive type PTCL. Nine patients had serological evidence suggesting active EBV infection. The clinical course after the onset of HS was generally fulminant in each group with a median survival of only 44d despite combination chemotherapy and/or empirical therapy with high-dose immunoglobulin and corticosteroids in six patients. In conclusion, HS represents a severe complication of EBV-PTCL. Although most patients develop HS at a time of active lymphoma, the syndrome may occur when the lymphoma is in remission. Because of the poor outcome, early diagnosis and a new modality of treatment for HS associated with EBV-PTCL should be pursued in future.