Angioimmunoblastic T cell lymphoma (AITL) with autoimmune thrombocytopenia

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  • SUEHIRO Satoru
    Department of Rheumatology and Medicine, Medical Institute of Bioregulation, Kyushu University
  • SHIRATSUCHI Motoaki
    Department of Rheumatology and Medicine, Medical Institute of Bioregulation, Kyushu University
  • SUEHIRO Youko
    Department of Rheumatology and Medicine, Medical Institute of Bioregulation, Kyushu University
  • OSHIMA Koichi
    First Department of Pathology, Fukuoka University
  • SHIOKAWA Satoshi
    Department of Rheumatology and Medicine, Medical Institute of Bioregulation, Kyushu University
  • NISHIMURA Junji
    Department of Rheumatology and Medicine, Medical Institute of Bioregulation, Kyushu University

Bibliographic Information

Other Title
  • 自己免疫性血小板減少症を伴った血管免疫芽球性T細胞リンパ腫
  • 症例 自己免疫性血小板減少症を伴った血管免疫芽球性T細胞リンパ腫
  • ショウレイ ジコ メンエキセイ ケッショウバン ゲンショウショウ オ トモナッタ ケッカン メンエキ ガキュウセイ Tサイボウ リンパ シュ

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Description

We present a case of angioimmunoblastic T cell lymphoma (AITL) with autoimmune thrombocytopenia. A 85-year-old man was admitted to our hospital with thrombocytopenia, generalized lymphadenopathy, pleural effusion, and splenomegaly in June 2000. Blood chemistry revealed hemoglobin and platelet counts of 8.8 g/dL and 26×109/L, respectively. The level of platelet-associate-IgG was 2568.9 ng/107 cells. The direct Coombs test was positive. The level of serum IL-6 was 10.2 pg/ml. Megakaryocytes in the bone marrow increased. Lymph node biopsy showed diffuse proliferation of atypical lymphoid cells with a clear cytoplasm accompanied by plasma cells and small vessels. He was diagnosed as having AITL with autoimmune thrombocytopenia and hemolytic anemia. He received repeated platelet transfusion, and a limited effect of prednisolone therapy on his platelet count was observed. Combination chemotherapy lessened the extent of the lymphadenopathy and slightly elongated the interval of platelet transfusion. We next performed splenic irradiation and a slight increase in the platelet count was observed. He died of pneumonia in August 2000. Autoimmune thrombocytopenia associated with AITL is rare and the therapy containing prednisolone and chemotherapy is reported to be partly effective. Our case showed a minor response of autoimmune thrombocytopenia to splenic irradiation. Therapeutic intervention for hypersplenism should be considered if thrombocytopenia is not improved by chemotherapy alone.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 43 (9), 841-845, 2002

    The Japanese Society of Hematology

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