Lymphoplasmacytic lymphoma with IgA hypergammaglobulinemia and liver involvement.

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.