Abstract. Angioimmunoblastic T cell lymphoma (AITL) is a peripheral T cell lymphoma, known to express CD3 and CD4, and, frequently, also CD10 and c-Maf-1. Hypergammaglobulinemia is not particularly rare in patients with AITL. However, AITL in conjunction with plasmacytosis in the peripheral blood is rare. The current report presents a case of CD10-negative AITL demonstrating leukemic change and plasmacytosis in the peripheral blood mimicking plasma cell leukemia. A 78-year-old male was admitted to hospital due to systemic lymph node enlargement, high serum IgG and IgA, and increased counts of plasmacytoid cells and lymphoid cells with atypical nuclei in the peripheral blood. Initially, plasma cell leukemia was suspected, due to the extreme increase in the number of plasma cells in the peripheral blood. However, the plasma cells did not show clonal expansion on examination by flow cytometry. Based on histological analyses, following a biopsy of an enlarged lymph node, the patient was diagnosed with AITL. This case suggests that when hypergammaglobulinemia and increases in B-lineage cells are observed, AITL should be considered in addition to disorders of B-lineage cells.
IntroductionAITL is the second most frequent subtype of peripheral T cell lymphoma in the Western world, accounting for ~25-30% of peripheral T cell lymphoma cases and 2-4% of all cases of lymphoma (1). It has been reported that AITL presents with a number of clinical symptoms, including fever, weight loss, chills, skin rashes, pruritis, lymphadenopathy, hepatosplenomegaly, anemia, thrombocytopenia and hypergammaglobulinemia (2). Patients with AITL, frequently exhibit anemia, thrombocytopenia and lymphopenia in the peripheral blood. However, it is rare for patients with AITL to have numerous plasma cells in the peripheral blood, which is a feature more typical of plasma cell leukemia (3-5). The current report presents a case of a patient with AITL, in whom leukemic change and plasmacytosis were observed in the peripheral blood, bone marrow and lymph nodes.
Case reportA 78-year-old male was admitted to hospital, due to systemic lymph node swelling and an elevated number of white blood cells in comparison with normal levels, which had been detected by his family doctor 10 days previously. Upon physical examination, lymph nodes in the cervical region, supraclavicular fossa, axillary fossa and inguinal fossa were found to be enlarged to 40 mm. The peripheral white blood cell count was elevated and numerous plasmacytoid cells without dysmorphic features, in addition to small-to-medium-sized lymphoid cells with atypical nuclei, were observed in his peripheral blood, as shown in Table I, Fig. 1 and Fig. 2A and B. 'Other' in the leukocyte classification in Table I, includes plasmacytoid cells (19% of whole white blood cells) and lymphoid cells with nuclear atypia (22% of whole white blood cells). Anemia and low platelet numbers were also observed.