成人慢性好中球減少症診療の参照ガイド  [in Japanese] Reference guide for adult chronic neutropenia  [in Japanese]

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Abstract

<p>慢性好中球減少症は好中球単独の減少が慢性的に持続する疾患群である。特に成人慢性好中球減少症は病態が不明な場合が多く,その稀少性からこれまで明確な診断基準や病型分類が作成されてこなかった。「成人慢性好中球減少症の診療ガイドライン作成に向けた予後追跡調査」研究班(日本医療研究開発機構研究費・難治性疾患実用化研究事業)では,診断基準・重症度分類・病型分類・治療方針等からなる「成人慢性好中球減少症診療の参照ガイド」を作成した。診断基準は好中球単独の減少(1,500/µ<i>l</i>未満)が3ヶ月以上続くこととし,好中球数に応じて重症度を分類した。また成人慢性好中球減少症の病因・病態を整理し病型分類を定め,それぞれの特徴を述べた。治療としては感染症対策や顆粒球コロニー刺激因子等の投与方針について述べた。成人慢性好中球減少症の診療の参考として,あるいは今後の研究の礎として,本ガイドが活用されることを期待する。</p>

<p>Chronic neutropenia is a rare hematological entity characterized by isolated neutropenia without anemia or thrombocytopenia. The diagnostic criteria and classification of chronic neutropenia have been more elusive in adult cases than pediatric cases. We herein propose the Reference Guide for Adult Chronic Neutropenia. In this guide, diagnosis of chronic neutropenia is based on recurrent or continuous neutropenia defined by absolute neutrophil count (ANC) less than 1,500/µ<i>l</i> for over three or more months. Severity of chronic neutropenia is defined as follows: severe, ANC <500/µ<i>l</i>; moderate, ANC between 500 and 1,000/µ<i>l</i>; mild, ANC between 1,000 and 1,500/µ<i>l</i>. In this guide, adult chronic neutropenia is classified into several types including autoimmune neutropenia, cyclic neutropenia, chronic neutropenia with T cell clone, and chronic idiopathic neutropenia with or without family history, and their clinical characteristics are described. Together with antibiotics, granulocyte colony-stimulating factors (G-CSFs) constitute the first-line treatment for adult chronic neutropenia patients at time of bacterial infections. Prophylactic use of G-CSFs or antibiotics is recommended only in selected clinical settings in severe cases.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 59(7), 845-857, 2018

    The Japanese Society of Hematology

Codes

  • NII Article ID (NAID)
    130007430620
  • NII NACSIS-CAT ID (NCID)
    AN00252940
  • Text Lang
    JPN
  • ISSN
    0485-1439
  • NDL Article ID
    029186340
  • NDL Call No.
    Z19-295
  • Data Source
    NDL  J-STAGE 
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