Successful Transplantation of the Thymus in Nezelof's Syndrome

Abstract

A 6-month-old girl with congenital thymic dysplasia or Nezelof's syndrome (lack of T cell function and normal levels of immunoglobulins) was given a transplant of a human thymus gland from a 14-week-old fetus and is surviving 36 months after transplant. Her clinical condition is the principal index of the success of the transplant since most in vitro and in vivo data indicate a full immunologic restoration has not been achieved. However, the number of E-rosettes in the peripheral blood has increased, the number of surface immunoglobulin-bearing cells has decreased, but there is little improvement in mitogen responsitivity. Immunoglobulin levels have increased with age, but there is no demonstrable specific antibody activity. Nevertheless, she remains free of infection, living in an unprotected environment.