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

Abstract Purpose Current knowledge of Lymphoma Associated Hemophagocytic Syndrome (LAHS) mainly relies on T/NK-cell LAHS cases, while B-cell lymphoma associated hemophagocytic syndrome (B-LAHS) variant are rare. We aimed at identifying a single-center cohort of patients with B-LAHS to shed light on relevant clinical and laboratory features, diagnosis, treatment and prognosis. Methods The clinical data of 36 patients with B-LAHS, admitted to the First Affiliated Hospital of Zhengzhou University in the time period ranging from January 2011 to May 2021 were analyzed retrospectively. Survival analysis was performed using the Kaplan–Meier method and groups were compared using the log-rank test. Results The 36 patients included 17 males and 19 females with a median age of 53.5 years (range: 28–74 years). Among these, the most common histopathological type diagnosed was diffuse large B-cell lymphoma (69%). The initial symptoms included fever (100%), splenomegaly (97%), multicavity effusion (83%), abnormal liver function (58%), and jaundice (44%). Patients treated with dexamethasone only seemed to have an inferior prognosis compared to those treated with etoposide-involving regimens (χ2 = 20.037, p < 0.001). Patients received based on the Hemophagocytic Lymphohistocytosis (HLH)-2004 protocol combined with multidrug chemotherapy had a significantly improved Overall Survival (OS) compared to patients who only underwent treatment according to the HLH-2004 regimen (χ2 = 30.744, p < 0.001). The 2-week mortality rate was 13.9% and the 1-year OS rate was 19.4%. Compared with the median survival time of 34 patients without Epstein-Barr virus (EBV) infection, 97 days (range: 24–322 days), two patients with EBV infection died within 8 days. Conclusion B-LAHS is relatively rare and has a high early mortality rate, mostly in middle-aged and elderly patients. The HLH-2004 regimen combined with multidrug chemotherapy is a reasonable choice for the treatment of B-LAHS, and EBV infection may be used as a reference indicator of a poor prognosis.