Lymphoma-associated hemophagocytic syndrome: An analysis of clinical features and survival of 27 cases from a single center in China / 肿瘤

ABSTRACT

Objective: To analyze the clinical features and treatment outcomes of patients with lymphoma-associated hemophagocytic syndrome (LAHS). Methods: Clinical data of 27 patients with LAHS diagnosed at Peking University Cancer Hospital between May 2007 and August 2014 were retrospectively analyzed. Results: Of the 27 patients, there were 18 males and 9 females, with a median age of 32 years (range 14 to 77). At diagnosis of lymphoma, 17 patients (63.0%) were stage HI/IV, 8 (29.6%) had Eastern Cooperative Oncology Group (ECOG) performance status (PS) score 5≥2, 12 (46.2%) had International Prognostic Index (IPI) score 5≥3. The most common subtype was extranodal natural killer/T cell lymphoma (ENKTCL) (74.1%, 20/27). Three patients presented with hemophagocytic syndrome (HPS) at lymphoma diagnosis, while the other 24 patients developed HPS during lymphoma progression after failure of chemotherapy. The clinical features of HPS were persistent fever (100.0%), splenomegaly (88.9%), hepatomegaly (37.0%), lymph node enlargement (63.0%), cytopenia (100.0%), ferritin increased (92.6%), hypertriglyceridemia (55.6%), hypofibrinogenemia (55.6%), and hemophagocytosis in bone marrow (70.4%). After a median follow-up of 11.0 months (range 0.3 to 66.0 months), 24 (88.9%) patients died, and 3 survived. The median overall survival (OS) after the diagnosis of lymphoma was 11 months, and the median OS after the diagnosis of HPS was 28 days. One patient receiving allogeneic hematopoietic stem cell transplantation (allo-HSCT) remained alive with complete remission for 53 months. Conclusion: The clinical manifestations of LAHS were complex, and the prognosis and survival time remain dismal. More effective therapeutic strategies should be develpoed, and allo-HSCT may provide survival benefts to LAHS.