Lymphopenic Agammaglobulinemia (Swiss Type) in Chicago

摘要: A LYMPHOPENIC form of congenital immunologic deficiency and thymic hypoplasia was first described by Glanzmann Riniker in 1950. 1 Subsequent detailed clinical, pathologic, studies siblings each the original two cases demonstrated familial nature this disorder associated defect agammaglobulinemia. 2,3 Since Swiss investigators have contributed heavily to definition autosomal recessive disease, it is frequently referred as "Swiss type" In contrast inheritance material, most descriptions lymphopenic hypogammaglobulinemia American literature indicated a sex-linked mode inheritance. On basis recent analyses Minneapolis previously reported from Boston Switzerland, apparent that diseases are separate entities. 4 Recently, number reports appeared concerning children with various atypical forms