Martín Mateos M A, Muñoz-López F, Cruz M
Department of Pediatrics, Hospital Clinico, University of Barcelona, Spain.
Allergol Immunopathol (Madr). 1988 Mar-Apr;16(2):113-9.
After presenting the case of a male patient who was diagnosed as suffering from the Wiskott-Aldrich syndrome at the age of 4 months, we review the main clinical (infections, bleeding and eczema), immunological (deficiency of IgM, normal IgG, elevated IgA and IgE, deficiency of total T lymphocytes, normal T4 levels, decreased levels of T8, diminished response to phytohemagglutinin and partial depletion of the lymphocyte population in biopsies of lymph nodes and spleen) and haematological (thrombocytopenia, dysmegakaryocytosis, decreased half-life and decreased granulation in the protoplasm of megakaryocytes) manifestations of this syndrome. In general, our aim is to carry out an up-to-date review of the syndrome, and to include comments on the diagnostic and therapeutic aspects.
在介绍了一名4个月大时被诊断患有维斯科特-奥尔德里奇综合征的男性患者的病例后，我们回顾了该综合征的主要临床（感染、出血和湿疹）、免疫学（IgM缺乏、IgG正常、IgA和IgE升高、总T淋巴细胞缺乏、T4水平正常、T8水平降低、对植物血凝素反应减弱以及淋巴结和脾脏活检中淋巴细胞群体部分耗竭）和血液学（血小板减少、巨核细胞发育异常、半衰期缩短和巨核细胞质内颗粒减少）表现。总体而言，我们的目的是对该综合征进行最新综述，并对诊断和治疗方面进行评论。
