| Abstract: | An immunity deficiency disease occurring in three siblings is described. Two siblings, a boy and a girl, died at ages 1½ years and 4½ years respectively with overwhelming varicells, varicella pneumonia and sepsis. Their disease included thymic hypoplasia, lymphopenia, deficient humoral and cellular immunity, absent serum IgA, neutropenia and eosinophilia. Transplantation of bone marrow, identical by cytotoxic and mixed leucocyte assay, red cell antigens and Gm and Inv factors was given to an affected girl from a normal sibling on two occasions. The first transplant given at 6 months of age resulted in clinical improvement of the patient and some evidence of immunologic reconstitution. Complete correction of the immunity defect was achieved following a second bone marrow transplant at 11 months of age. A delayed onset and prolonged course of GVH reaction was observed following the second transplant. The patient survived the GVH without specific therapy. Evidence for complete immunologic reconstitution continued to be present 1 year following the second transplantation. |
