Exacerbation of autoimmune neutropenia to agranulocytosis in association with severe autoimmune thrombocytopenia and hemolytic anemia in a patient with Sjögren's syndrome

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  • SHIMOJI Sonoko
    Departments of Hematology and Clinical Immunology, Kobe City Medical Center, General Hospital
  • TAKIUCHI Yohko
    Departments of Hematology and Clinical Immunology, Kobe City Medical Center, General Hospital
  • MARUOKA Hayato
    Department of Laboratory Medicine, Kobe City Medical Center, General Hospital
  • INOUE Daichi
    Departments of Hematology and Clinical Immunology, Kobe City Medical Center, General Hospital
  • KIMURA Takaharu
    Departments of Hematology and Clinical Immunology, Kobe City Medical Center, General Hospital
  • MORI Minako
    Departments of Hematology and Clinical Immunology, Kobe City Medical Center, General Hospital
  • NAGAI Yuya
    Departments of Hematology and Clinical Immunology, Kobe City Medical Center, General Hospital
  • TOGAMI Katuhiro
    Departments of Hematology and Clinical Immunology, Kobe City Medical Center, General Hospital
  • TABATA Sumie
    Departments of Hematology and Clinical Immunology, Kobe City Medical Center, General Hospital
  • KURATA Masayuki
    Departments of Hematology and Clinical Immunology, Kobe City Medical Center, General Hospital
  • MATSUSHITA Akiko
    Departments of Hematology and Clinical Immunology, Kobe City Medical Center, General Hospital
  • NAGAI Kenichi
    Departments of Hematology and Clinical Immunology, Kobe City Medical Center, General Hospital
  • TAKAHASHI Takayuki
    Departments of Hematology and Clinical Immunology, Kobe City Medical Center, General Hospital

Bibliographic Information

Other Title
  • シェーグレン症候群を背景として自己免疫性血小板減少症および溶血性貧血を伴い，無顆粒球症に進展した自己免疫性好中球減少症
  • 症例報告 シェーグレン症候群を背景として自己免疫性血小板減少症および溶血性貧血を伴い,無顆粒球症に進展した自己免疫性好中球減少症
  • ショウレイ ホウコク シェーグレン ショウコウグン オ ハイケイ ト シテ ジコ メンエキセイ ケッショウバン ゲンショウショウ オヨビ ヨウケツセイ ヒンケツ オ トモナイ ムカリュウキュウショウ ニ シンテン シタ ジコ メンエキセイ コウ チュウキュウ ゲンショウショウ

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Abstract

A 73-year-old woman with Sjögren's syndrome and autoimmune neutropenia (AIN) associated with large granular lymphocytosis of the polyclonal T cell type, demonstrated autoimmune thrombocytopenia (AIT) at diagnosis of sigmoid colon cancer. Ten months later, both AIN and AIT had exacerbated to agranulocytosis and severe thrombocytopenia below 10×109/L, respectively. There were no dysplastic features of bone marrow hematopoietic cells. Furthermore, an in vitro assay of hematopoietic progenitors showed normal granuloid and erythroid colony formation. Although we serially treated her with prednisolone (oral), filgrastim, intravenous high-dose immunoglobulin infusion, cyclophosphamide (oral), danazol, cyclosporine A (oral), and rituximab, number of neutrophils and platelets elevated only temporarily. During the course of agranulocytosis and severe thrombocytopenia, the patient also developed autoimmune hemolytic anemia (AIHA). She died of pneumonia 5 months after the onset of agranulocytosis. This case is very unique and novel in terms of autoimmune phenomena simultaneously directed to granulocytes, platelets, and red blood cells under the background of Sjögren's syndrome.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 52 (7), 535-539, 2011

    The Japanese Society of Hematology

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