J Med Assoc Thai 2009; 92 (6):65

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Lymphoplasmacytic Lymphoma with IgA Hypergammaglobulinemia and Liver Involvement
Polprasert C Mail, Wongchitrat C , Assanasen T , Prayongratana K

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

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