21才で腎不全により死亡したWiskott-Aldrich症候群の1剖検例

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タイトル別名
  • An Autopsy Case of Wiskott-Aldrich Syndrome in a 21-year-old Male with Renal Failure
  • 21サイ デ ジンフゼン ニ ヨリ シボウシタ Wiskott Aldrich

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An autopsy case of Wiskott-Aldrich syndrome in a 21-year-old male was reported. His death was due to renal failure and digestive tract bleeding. He had suffered from repeated infections, eczema and bleeding tendency since 6 months after birth. The prolonged survive of this patient might be due to deligent management rather than due to a mild form of the disease because he had severe recurrent infections and bleeding tendency during the active clinical course.<br>The family history revealed that a younger brother of his mother had died of similar syndrome in infancy.<br>Laboratory data during the last admission showed marked thrombocytopenia, slight lymphocytopenia and eosinophilia. Delayed hypersensitivity responses tested with PPD and DNCB were absent. The in-vitro blastogenic responses of lymphocytes by PHA and Con A were decreased. The level of IgM was decreased to the lower limit of the normal range but IgE level was normal. There was a marked deficiency of isohemagglutinin and of the ability to form antibodies against typhoid and paratyphoid vaccine. E-rossete forming cells were also decreased.<br>The histological findings at autopsy showed generalized depletion of lymphocytes in the lymphnodes including paracortical regions. Macroscopically the kidneys were small and firm with finely granulated surfaces. Microscopically numerous glomeruli were the sites of intense scarring and extensive tubular atrophy was present supporting histological diagnosis of chronic glomerulonephritis. There were marked degree of interstitial fibrosis and the infiltration of small round cells.

収録刊行物

  • 臨床血液

    臨床血液 19 (12), 1665-1671, 1978

    一般社団法人 日本血液学会

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