[New data on thymomegaly as a syndrome of congenital (primary) immunodeficiency]

Basing on a combined clinicomorphological investigation of the thymicolymphatic and neuroendocrine systems including measurements of blood thymic hormones and their levels in thymic tissue it is shown that congenital thymomegaly can be considered a dysfunction of the hypothalamo-hypophysial system. The dysfunction manifests with polyglandular endocrinopathy and congenital immunodeficiency related to the group of thymus-dependent immunodeficiencies. Thymomegaly is often coupled with congenital malformations.