Objective: To investigate the clinical and laboratory features of patient with B cell lymphoma associated hemophagocytic syndrome(B-LAHS).
Methods: The clinical data of 10 cases of B-LAHS were retrospectively analysed and the relevant literatures were reviewed.
Results: The median age of 10 cases diagnosed as B-LAHS was 55.5 (31-88) years old, and median time from attack to diagnosis was 2 months (2 weeks-4 months). The diagnosis can be made histopathologically and immunohistochemically by bone marrow biopsy. Among them 7 cases were diagnosed as large B cell lymphoma, 2 cases as mantle cell lymphoma and 1 case as small B cell lymphoma. The prominent clinical symptoms and signs were persistent fever (100%) and splenomegaly(90%), and the involvements with respiratory and digestive system were common. Another 1 case had systemic muscle pain and lactic acidosis as the first onset. Laboratory studies showed hepatic dysfunction, significantly elevated ferritin and lactate dehydrogenase, abnormal lymphocytes in peripheral blood smear, and hemophagocytosis in bone marrow smear. The FSC/SSC abnormalities of cloned B lymphoma cells were detected through flow cytometry (FCM). The complete remission (CR) was maintained in 4 cases receiving immunochemotherapy based on rituximab.
Conclusion: B-LAHS possesses heterogeneous clinical manifestations and rapid deterioration. Bone marrow biopsy and immunohistochemical examination can confirm the diagnosis. FCM may improve the early diagnosis of B-LAHS.