Study on Treatment of Lymphoma-Associated Hemophagocytic Syndrome with Fludarabine Combined with Methylprednisolone and Globulin

Hemophagocytic syndrome (HPS) is a life-threatening condition of severe hyperinflammation caused by the uncontrolled proliferation of activated lymphocytes and histiocytes secreting high amounts of inflammatory cytokines. Lymphoma-associated hemophagocytic syndrome (LAHS) clinically featured by histiocytic proliferation, fever, hepatosplenomegaly, cytopenia, deranged liver function and frequent coagulopathy. The immediate aim of therapy in LAHS is suppression of the severe hyperinflammation, which can be achieved with immunosuppressive/immunomodulatary agents and cytostatic drugs. In this study, we diagnosed 11 cases of LAHS according to HLH-2004 diagnostic criteria in Beijing Friendship Hospital during the period from June 2005 to Jan 2008, and collected informations about the patients with LAHS: primary diseases, manifestions, laboratory findings and survival. 2 cases large B cell lymphoma, 1 case mantle lymphoma, 4 cases extranodal NK/T cell lymphoma, 3 cases peripheral T cell lymphoma and 1 case angioimmunoblastic T-cell lymphoma were included in this research. The median age was 49 years, and the range was 33–74 years. 7 out of 11 were female and 4 were male. All the 11 patients received regimens containing fludarabine (25mg/m²/d, d1–5), methylprednisolone (2mg/kg/d, d1–14, 1mg/kg/d, d15–28) and immunoglobulin (0.5g/kg/d, d1–14) after finally diagnosed. 7 of the 11 patients were markedly improved after treatment and then received lymphoma chemotherapy. The median survival time had reached to 1.4 year. The other 4 aggravated. This outcome was much better than what A-Reum Han had reported (