Abstract

An otherwise healthy term newborn received non-irradiated platelets in the first day of life for isoimmune thrombocytopenia. At 10 days of age the child developed an ultimately fatal disease characterized by rash, pneumonitis and bone marrow depression. Although no peripheral lymphocyte chimerism was detected, skin biopsy revealed a vacuolated basal cell layer with pyknotic nuclei, dermal layer lymphocytic infiltrate and edema, epidermal spongiosis, occasional infarcts, and many eosinophilic bodies with satellitosis. Bone marrow biopsy and autopsy findings were also most consistent with GVH. Immunologic evaluation revealed an adequate number of neutrophils. Complement levels were normal. The child's IgM and IgE levels were normal and her B cells could produce IgM after PWM stimulation. Peripheral lymphocytes had both maternal and paternal HLA antigens. Rosette techniques revealed 40% T cells and 10% B cells, both within the low normal range for age. In the one way MLR, the patient's cells responded well to various donors except to the mother. PHA stimulation was also within normal range. Thus there was no evidence to suggest a defect in the child's immune system. The appearance of a GVH reaction in an apparently immunocompetent infant raises the question of irradiating all blood products containing viable lymphocytes before administration to newborns.

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