Lymphoplasmacytic Lymphoma/ Waldenström Macroglobulinemia in a Patient with Hemophagocytic Syndrome

Abstract Waldenström macroglobulinemia (WM) is a lymphoplasmacytic lymphoma (LPL) associated with monoclonal immunoglobulin M protein (IgM). It is a rare type of indolent B-cell lymphoma. MYD88L265P mutation is an important molecular marker for the diagnosis and the differential diagnosis of WM. Hemophagocytic lymphohistiocytosis (HLH) is a severe inflammatory response syndrome caused by genetic or acquired immunoregulatory dysfunction. It is extremely rare for WM and HLH to occur simultaneously in the same patient. So far, no case of WM and HLH has been reported, and the reason for the coexistence of WM and HLH is still unclear. With the advent of new therapies, the survival time of patients with WM and HLH has improved. Therefore, it is of great value to further investigate the clinical features, pathophysiology and prognosis of these cases. We present a case study of a 68-year-old woman who was diagnosed with WM. Following treatment with the Bendamustine/rituximab (BR) regimen, the patient experienced persistent high fever, leading to further investigations for a diagnosis of HLH. This report highlights the rarity of WM combined with HLH and provides valuable raw data for future clinical research.