ABSTRACT
Objective To explore the features of non-Hodgkin lymphoma (NHL) with hemophagocytic syndrome (HPS) as the initial manifestation at diagnosis. Methods The morbidity and treatment of two B-cell NHL patients with HPS as the initial manifestation were retrospectively analyzed, and the literature were reviewed at home and abroad. Results Two B-cell NHL patients with HPS were treated with the HLH-94 protocol to control febrile condition. After then, R-CHOP protocol (dexamethasone substituted for prednisone) was used and their results were good. In reviewing the literature, it was felt that the main sub-type of B-cell lymphoma-associated HPS (B-LAHPS) was diffuse large B-cell lymphoma. However, sub-types of T-cell lymphoma-associated HPS (T-LAHPS) had no significant difference. The incidence of T-LAHPS was significantly higher than that of B-LAHPS in China and South Korea, but there was no significant difference in the Western countries. T-cell lymphomas were more vulnerable to HPS. The median age of B-LAHPS patients at diagnosis was significantly higher than that of T-LAHPS patients. The median survival and overall survival time of B-LAHPS patients were longer than those of T-LAHPS patients by virtue of the application of rituximab. Conclusion B-cell NHL with HPS as the initial manifestation treated with the 2004-HLH plus R-CHOP protocol could achieve good outcomes.