Angioimmunoblastic T-cell lymphoma with marked polyclonal plasmacytosis in peripheral blood and bone marrow mimicking plasma cell leukemia

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Other Title
  • 形質細胞性白血病を疑わせるほど末梢血および骨髄に著明な多クローン性形質細胞増生を伴ったAngioimmunoblastic T-cell lymphoma
  • 症例報告 形質細胞性白血病を疑わせるほど末梢血および骨髄に著明な多クローン性形質細胞増生を伴ったAngioimmunoblastic T-cell lymphoma
  • ショウレイ ホウコク ケイシツ サイボウセイ ハッケツビョウ オ ウタガワセル ホド マッショウケツ オヨビ コツズイ ニ チョメイ ナ タクローンセイ ケイシツ サイボウ ゾウセイ オ トモナッタ Angioimmunoblastic T cell lymphoma

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Abstract

A 70-year-old man was admitted to our hospital with fever, generalized lymphadenopathy and hypoxia in October 2009. Blood examination demonstrated leukocytosis, anemia, thrombocytopenia and hyper γ-globulinemia. Peripheral blood and bone marrow smear showed marked plasma cell proliferation mimicking plasma cell leukemia. However, flow cytometric analysis showed that plasma cells were of polyclonal origin and M-protein was not detected by immunofixation of serum protein. Elevations of soluble interleukin 2 receptor and serum IL-6 were observed. A heavy Epstein-Barr viral load was detected in serum by real-time PCR. Biopsy was obtained from the right inguinal lymph node. The pathological diagnosis was angioimmunoblastic T-cell lymphoma (AITL) and rearrangement of the T-cell receptor Cβ1 gene was detected. The patient was treated with CHOP therapy, and all clinical manifestations, including fever, lymphadenopathy, anemia, thrombocytopenia, hyper γ-globulinemia, plasmacytosis and hypoxia, were improved. Only a few reported cases have demonstrated AITL with marked polyclonal plasmacytosis. Although pathological mechanisms of plasmacytosis in AITL patients have not been fully elucidated, it is suggested that IL-6 and IL-10 were involved in its pathogenesis in the present case.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 52 (7), 563-569, 2011

    The Japanese Society of Hematology

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