Successful rituximab treatment for acquired amegakaryocytic thrombocytopenic purpura complicated with Coombs-negative autoimmune hemolytic anemia

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  • HASHIMOTO Akari
    Department of Hematology and Oncology, Oji General Hospital
  • FUJIMI Akihito
    Department of Hematology and Oncology, Oji General Hospital
  • KANISAWA Yuji
    Department of Hematology and Oncology, Oji General Hospital
  • MATSUNO Teppei
    Department of Gastroenterology, Oji General Hospital
  • OKUDA Toshinori
    Department of Gastroenterology, Oji General Hospital
  • MINAMI Shinya
    Department of Gastroenterology, Oji General Hospital
  • DOI Tadashi
    Department of Gastroenterology, Oji General Hospital
  • ISHIKAWA Kazuma
    4th department of Internal Medicine, School of Medicine, Sapporo Medical University
  • UEMURA Naoki
    4th department of Internal Medicine, School of Medicine, Sapporo Medical University
  • TOMARU Utano
    Department of pathology, Hokkaido University Graduate School of Medicine

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Other Title
  • クームス陰性自己免疫性溶血性貧血を併発しリツキシマブが奏効した後天性無巨核球性血小板減少性紫斑病
  • 症例報告 クームス陰性自己免疫性溶血性貧血を併発しリツキシマブが奏効した後天性無巨核球性血小板減少性紫斑病
  • ショウレイ ホウコク クームス インセイ ジコ メンエキセイ ヨウケツセイ ヒンケツ オ ヘイハツ シ リツキシマブ ガ ソウコウ シタ コウテンセイ ムキョカクキュウセイ ケッショウバン ゲンショウセイ シハンビョウ

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Abstract

Acquired amegakaryocytic thrombocytopenic purpura (AATP) is a rare disorder characterized by severe thrombocytopenia associated with total absence or a selective decrease in bone marrow megakaryocytes. A 67-year-old male presented with a 2-month bleeding tendency. He was referred to our hospital because of severe thrombocytopenia. Bone marrow biopsy showed complete absence of megakaryocytes without dysplasia in cells of the myeloid and erythroid lineages. AATP was diagnosed. In addition, mild normocytic normochromic anemia and reticulocytosis were also observed and haptoglobin was below the detectable level. Coombs-negative autoimmune hemolytic anemia (AIHA) was diagnosed based on the high titer of RBC-bound IgG and negative direct and indirect coombs test results. He was first treated with cyclosporine 200 mg per day and subsequently with prednisolone but only slight temporary improvement was achieved. Administration of eight doses of rituximab 375 mg/m2 per week ameliorated both thrombocytopenia and anemia. AATP should be considered in the differential diagnosis of thrombocytopenia, and immunosuppressive therapy is a potential first-line treatment. This is the first case report of AATP accompanied by AIHA successfully treated with rituximab.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 54 (6), 568-573, 2013

    The Japanese Society of Hematology

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