t(11;18)(q21;q21)-positive Advanced-stage MALT Lymphoma Associated with Monoclonal Gammopathy : Resistance to Rituximab or Rituximab-containing Chemotherapy

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Here we describe two cases of mucosa-associated lymphoid tissue (MALT) lymphoma with monoclonal immunoglobulins (Igs). The first case was a 77-year-old man with primary lymphoma of the lung. Immunoelectrophoresis detected IgM-κ in serum and κ light chain excretion into urine. Three months after treatment with single-agent rituximab, a large amount of pleural fluid was found to have accumulated. The fluid contained CD5-, CD10-, CD19+, CD38+ and CD138-/+ lymphoma cells with lymphoplasmacytoid appearance. Although a small fraction of the cells were CD20+, the majority of the lymphoma cells were negative and expressed surface-membrane IgM-κ at low levels. The cells possessed a karyotype of 46, XY, t(11;18)(q21;q21). The second case was a 55-year-old man who underwent total gastrectomy due to gastric perforation. Surgical specimens demonstrated the histopathological features of MALT lymphoma associated with plasma cell differentiation. The lymphoma cells had a 46, XY, t(11;18)(q21;q21) karyotype. Monoclonal Igs detected were serum IgA (M)-κ and urinary κ light chain. The patient was subsequently treated with six cycles of R-CVP (rituximab, cyclophosphamide, vincristine and prednisolone) ; however, serum monoclonal Ig levels were not affected. The lymphoma cells in both cases may have contained two populations, a rituximab-sensitive CD20+ population and a rituximab-resistant population that had differentiated into the Ig-secreting plasma cell stage. [J Clin Exp Hematopathol 48(2) : 47-54, 2008]

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