Okuda et al. reported a case of successful treatment for severe aplastic anemia (AA) concurrent with lymphoplasmacytic lymphoma/Waldenstrm macroglobulinemia (LPL/ WM) (1).This article sheds light on a complex situation regarding the treatment for AA concurrent with lymphoma, including LPL/WM, for which there is still no consensus recommendation.LPL/WM is a rare entity of indolent-B-cell lymphoma, characterized by the infiltration of neoplastic B-cells with plasmacytic differentiation into the bone marrow, accompanied by immunoglobulin (Ig) monoclonal gammopathy (2, 3).The 5th edition of the World Health Organization Classification of Hematolymphoid Tumors divided LPL into two subtypes: IgM-producing LPL (WM, -95%) and non-IgM type LPL (up to 5%).The IgM paraprotein can cause not only hyperviscosity syndrome, but also autoantibodies, resulting in a variety of autoimmune phenomena, including secondary cold agglutinin disease, thrombocytopenia, and von Willebrand disease.Publications regarding AA concurrent with lymphoma are limited (4-6), and the etiology of the concurrency varies.If AA and lymphoma are diagnosed simultaneously, then AA is generally considered to be a paraneoplastic autoimmune phenomenon of lymphoma.However, according to reports from the European Society of Blood and Bone Marrow Transplantation, treatment for lymphoma does not necessarily resolve AA, even if the lymphoma achieves a complete response (4).Thus, no consensus treatment recommendation for AA concurrent with lymphoma has yet been established, and the treatment should be determined on an individual basis.
Naohiro Sekiguchi (Thu,) studied this question.