Leukemic-phase mucosa-associated lymphoid tissue lymphoma

A 78-year-old man presenting with leukocytosis was admitted to our hospital. The patient was asymptomatic and showed no lymphadenopathy. Peripheral blood flow cytometry revealed a leukemic-phase B-cell lymphoma with medium-to-large abnormal cells with reticulum. Positron emission tomography/computed tomography revealed abnormal uptake in the right orbit, bone marrow, and spleen. We performed immunological staining and fluorescence in situ hybridization on tissues extracted from the right orbit and bone marrow, which led to the diagnosis of mucosa-associated lymphoid tissue (MALT) lymphoma. Polymerase chain reaction analysis of immunoglobulin heavy chain rearrangements in the right orbital mass and bone marrow suggested that they were identical clones. Based on these collective findings, the diagnosis of leukemic-phase MALT lymphoma was confirmed, with sites of involvement including the bone marrow, peripheral blood, right orbit, and spleen. This is a highly rare case of leukemic MALT lymphoma.