Chronic Neutropenia during Childhood

  • SATO Takashi
    Department of Pediatric Hematology, Hiroshima University Hospital
  • KOBAYASHI Masao
    Department of Pediatric Hematology, Hiroshima University Hospital

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  • 小児慢性好中球減少症

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Abstract

Chronic neutropenia during childhood involves various underlying conditions. As an external cause factor, infection, administered medicines, bone marrow replacement by malignant cells, allo-or auto-antibodies that cause most chronic neutropenia in infancy, etc., should be considered. Although, the chronic neutropenia caused by intrinsic factors, such as severe congenital neutropenia, are rare, the clinical manifestations are severe. The approach to the diagnostic evaluation of a patient can be guided largely by clinical history and physical examination and does not always require an extensive laboratory evaluation. Recent advances in this field showed several molecular abnormalities in chronic neutropenia caused by intrinsic factors. However, there exist discrepancies between the molecular abnormalities and the clinical manifestations. Further investigations are required to elucidate the precise mechanisms of the disease. In this article, we show clinical manifestations, bone marrow findings, and molecular abnormalities of some representative diseases that cause chronic neutropenia during childhood.

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