AN AUTOPSY CASE OF CONGENITAL HYPOGAMMAGLOBULINEMIA IN TWO BROTHERS

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  • 家族性発生をみた先天性低γグロブリン血症の1剖検例
  • カゾクセイハツセイ オ ミタ センテンセイ テイガンマ グロブリン ケツショウ ノ 1 ボウケンレイ

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1. This report deals with an autopsy case of congenital hypogammaglobulinemia in three month male infant.<BR>2. This case is consistent with Swiss type hypogammaglobulinemia in consequence of pathologic studies. It is recognized marked hypoplasia in general lymphoid tissues such as thymus, lymph nodes, spleen and tonsil except ileum and appendix. Histologic examination reveals that thymus is devoid of small Iymphocytes, with poorly developed lobules, no differentiation in the cortex and medulla, and no Hassall's corpuscles. Lymph node shows a marked paucity of small lymphocytes in both the cortex and medulla, and no germinal follicles and no plasma cells are present.<BR>3. His brother's histopathologic features reported previously by Yoshimura et al are extremely similar to this case. It is considered Swiss type hypogammaglobulinemia occurred in two brothers owing to investigation of their pathologicandhistologic data of lymphoid tissues especially thymus and lymph nodes.<BR>4. It is presumed viral infection in late stage because of histologic pictures of the heart and lung.

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