Lymphoplasmacytic Lymphoma with IgA Hypergammaglobulinemia and Liver Involvement
Chantana Polprasert MD*, Chatchawan Wongchitrat MD**, Thamathorn Assanasen MD*** , Kannadit Prayongratana MD*
Affiliation : * Division of Hematology, Department of Medicine, Srinakarinwirot University, Bangkok, Thailand ** Division of Gastroenterology, Department of Medicine, Srinakarinwirot University, Bangkok, Thailand *** Department of Pathology, Chulalongkorn University, Bangkok, Thailand
Background : Lymphoplasmacytic lymphoma (LPL) is a low grade lymphoma. Most cases are Waldenstorm
macroglobulinemia which has IgM hypergammaglobulinemia. Lymphoplasmacytic lymphoma with IgA
hypergammaglobulinemia is less than 5%. Liver involvement was reported in 20%. However this disease has
been found to be mostly presented with lymphadenopathy and hypergammaglobulinemia.
Case Report : We present a forty-year-old woman with anemia, renal insufficiency and abnormal liver function
test. Liver biopsy showed atypical clonal B-cell lymphoproliferation, small cells with prominent plasmacytic
differentiation. Serum protein electrophoresis showed monoclonal gammopathy which was IgA. Rituximab,
fludarabine and cyclophosphamide were given and resulting in partial response.
Conclusion : The presentation of LPL can mimic multiple myeloma (anemia, renal failure and monoclonal
gammopathy). Definite histological and immunological technique should be done to confirm the diagnosis.
Keywords : Hypergammaglobulinemia, Immunoglobulin A, Liver diseases, Waldenstrom macroglobulinemia
