Adult Lymphoma-Associated Hemophagocytic Syndrome Is a Life-Threatening Disease: Retrospective 10-Year Review of 89 Cases at a Single Center

Lymphoma is classified into Hodgkin's and non-Hodgkin's lymphoma (B- cell or T-cell type). Lymphoma-associated hemophagocytic syndrome (LAHS) is a life-threatening disease with dismal prognosis. To assess the hemophagocytic lymphohistiocytosis (HLH)-related clinical features and prognosis in the immunophenotype of LAHS, we retrospectively evaluated 483 patients with HLH presented at our institution between December 2006 and March 2016. Eighty-nine adult patients were diagnosed with LAHS. Of those, 2 cases (2.2%) were Hodgkin's lymphoma with Epstein-Barr virus infection. Eighty-seven cases (97.8%) were non-Hodgkin's lymphoma consisting 25 cases (28.7%) of B-cell LAHS (B-LAHS) and 62 cases (71.3%) of T-cell LAHS (T-LAHS). There were 17 male and 8 female patients in B-LAHS with a median age of 57 years. There were 40 male and 22 female patients in T-LAHS with a median age of 39 years. T-LAHS patients were associated with younger age which was significantly different with that in B-LAHS patients (p=0.000). Clinical features were summarized according to HLH-2004 diagnosis criteria. For B-LAHS, fever and elevated serum ferritin exhibited in 100% of cases; elevated sCD25 was found in 94.7%; decreased NK cell activity was identified in 42.1%; pancytopenia was found in 80.0%; hemophagocytosis was found in 80.0%; splenomegaly presented in 92.0%; hypofibrinogenemia was found in 20.0%; hypertriglyceridemia presented in 26.1%. For T-LAHS, fever exhibited in 98.4%; elevated serum ferritin was found in 96.3%; elevated sCD25 presented in 87.8%; decreased NK cell activity was identified in 51.3%; pancytopenia exhibited in 80.6%; hemophagocytosis was found in 84.2%; splenomegaly presented in 85.4%; hypofibrinogenemia was found in 59.3%; hypertriglyceridemia presented in 50.0%. The 1-month, 3-month, and 6-month overall survival rate was 88.0%, 67.6%, and 53.1% in B-LAHS patients versus 54.5%, 28.2%, and 18.8% in T-LAHS patients. Ten T-LAHS patients underwent allogeneic hematopoietic stem cell transplantation (HSCT) with survival time of 1, 3, 4, 5, 5, 6, 7, 52, 84, 113 months, respectively. Nine patients with T-LAHS (14.5%) survived up to the end of follow-up, of which 6 patients (66.7%) received HSCT and 3 patients (33.3%) were without transplantation. Our results indicated the HLH-related clinical features present differently between T-LAHS and B-LAHS. Compared with B-LAHS, T-LAHS associate with earlier onset and inferior prognosis. In addition, allogenic HSCT might be an effective treatment approach for T-LAHS patients to achieve long-term survival.