Clinical Features and Prognosis of B-cell Lymphoma-associated Hemophagocytic Syndrome: A Retrospective Single Center Study

Abstract

Secondary Hemophagocytic Syndrome (HPS), also known as Hemophagocytic Lymphohistocytosis (HLH), is a life-threatening syndrome caused by secondary overstimulation of the immune system, with a high mortality rate even after appropriate treatment. HLH is often triggered by malignancies, infections or autoimmune diseases with the Malignancy-Associated Hemophagocytic Syndrome (MAHS) accounting for the highest proportion of secondary HLH (about 48%), and Lymphoma Associated Hemophagocytic Syndrome (LAHS) being the most common [1–3]. Among the LAHS cases, T/NK-cell lymphoma is much more common than the rarely seen B-cell lymphoma [4]. B-cell LAHS (B-LAHS) is predominantly described in Asian populations but larger sets are rare. Early clinical manifestations of HLH are nonspecific, mostly manifesting in persistent fever, pancytopenia and hepatosplenomegaly with an aggressive disease progress [5]. Therefore, early diagnosis and immediate introduction of appropriate treatment are crucial for these patients.