Abstract
Two sisters from one family with a selective cellular immunodeficiency are described. The first girl born was admitted to the hospital at 10 months of age with generalized vaccinia following smallpox vaccination. Thymus-dependent immunity could not be demonstrated, whereas humoral immunity was present. She was treated with several thymus transplants and transfer factor injections, resulting in healing of the vaccinia lesions and partial, but transient, restoration of the thymus-dependent immunity. In spite of this success she developed a lymphosarcoma and died at the age of 3 years. Her younger sister was admitted at the age of 1.5 years and was suffering then from a fatal graft-versus-host reaction following a blood transfusion given elsewhere. Clinical and immunological findings of the two patients are presented in detail.
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