Bone marrow cells from a patient with severe combined immunodeficiency were studied in vitro for thymus-dependent lymphocyte (T cell) Severe combined immunodeficiency disease (SCID) is a diagnostic term for a heterogeneous group of congenital disorders usually ascribed to a defect in the development or function of lymphoid stem cells (1). Such patients usually have defective development and function of both thymus-dependent lymphocyte (T cell) and thymus-independent lymphocyte (B cell) populations (2). Immunologic reconstitution in patients with this disease has been repeatedly achieved after transplantation of marrow from related, histocompatible donors (3)(4)(5)(6)(7)(8) In this report, we present evidence to indicate that, in certain variants of SCID, transplantation of both fetal liver and thymus may be necessary to provide both the stem cell and an inductive influence necessary for immunologic reconstitution.
MATERIALS AND METHODSCase Report. The patient, a 20-month-old white boy, was a full-term 3.8-kg product of a normal pregnancy. Growth and development were normal until age 10 weeks, at which time a monilial rash was first noted behind his right ear; this was followed by severe oral moniliasis at age 4 months. Both conditions were refractory to antifungal therapy. At 5 months of age, poor feeding and progressive weight loss developed. On Sept., 18, 1975, at age 7 months, he was admitted to our hospital.At the time of admission, the patient was markedly cachectic, weighed 5.5 kg, and had severe moniliasis involving the oral cavity and perineal area. Clinical and roentgenographic evidence of interstitial pneumonia, subsequently documented to be due to Pneumocystis carinii, was noted. No