The Solitary Kostamann's Agranulocytosis in a 16 Year-Old Male

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  • TSUKAMOTO Norifumi
    Third Department of Internal Medicine, Gunma University School of Medicine
  • WAKAMATSU Eri
    Third Department of Internal Medicine, Gunma University School of Medicine
  • IZUMI Tomoyuki
    Third Department of Internal Medicine, Gunma University School of Medicine
  • NARAHARA Nobuhiro
    Third Department of Internal Medicine, Gunma University School of Medicine
  • YAMAUCHI Hiromasa
    Third Department of Internal Medicine, Gunma University School of Medicine
  • NOGIWA Eishi
    Third Department of Internal Medicine, Gunma University School of Medicine
  • OMINE Mitsuhiro
    Third Department of Internal Medicine, Gunma University School of Medicine
  • MAEKAWA Tadashi
    Third Department of Internal Medicine, Gunma University School of Medicine

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Other Title
  • Kostmann型無顆粒球症の16歳男子孤発例

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Description

A 16 year-old male was admitted because of a marked granulocytopenia associated with delayed wound healing. He has had a history of increased susceptibility to bacterial infection since infancy, and leukopenia had been pointed out repeatedly. Family study revealed neither consanguinity nor hematological abnormalites in his pedigree.<br>He was physically unremarkable except a mild lymphadenopathy and palpable liver. His peripheral blood showed leukopenia (3,300/μl) with nearly complete absence of mature neutophils, and significant increase in the number of both monocytes and eosinophils. Maturation arrest at the promyelocyte stage was the most prominent feature of his bone marrow smear. The marrow granulocyte reserve and marginal granulocyte pool were greatly diminished. The numbers of CFU-C were normal, but the colonies were prominently of macrophage lineage. Increased monocyte count may explain his normal developement and nearly uneventul life up to the adolescence.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 27 (7), 1080-1084, 1986

    The Japanese Society of Hematology

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