Reversal of enterocolitis-associated combined immunodeficiency by plasma therapy.
Two 6-month-old male infants with diarrhea, malabsorption, and hypoproteinemia, who were initially diagnosed as having combined immunodeficiency syndrome, recovered with intensive plasma therapy. Prior to the onset of diarrhea, they had normal serum protein and lymphocyte values. Immunologic features of combined immunodeficiency included lymphopenia, diminished B and T cells, cutaneous anergy, low immunoglobulin levels, and poor lymphocyte proliferative responses in vitro. Prior to therapy, both children had rectal ulcerations by proctosigmoidoscopy, colitis by rectal biopsy, and moderate to severe intestinal villus abnormalities by small bowel biopsy; plasma cells were absent Both had generalized malabsorption of all nutrients. Both infants were given irradiated fresh-frozen plasma for one to two months at 11 to 20 ml/kg/day to replace intestinal protein losses. During this time, diarrhea slowed, biopsy morphology improved, and immunoglobulin levels and T-cell function became normal. After discontinuance of plasma therapy, normal immune function and a normal stool pattern with reversal of malabsorption continued. Since intensive plasma therapy may have contributed to the reversal of the immunodeficiency state, a trial of such therapy is recommended in similar patients.
D001402:B-Lymphocytes / Q000032:analysis; D001803:Blood Transfusion; D004761:Enterocolitis, Pseudomembranous / Q000150:complications / Q000473:pathology / Q000628:therapy*; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis; D007153:Immunologic Deficiency Syndromes / Q000209:etiology / Q000628:therapy*; D007223:Infant; D007421:Intestine, Small / Q000473:pathology; D008297:Male; D010949:Plasma; D012007:Rectum / Q000473:pathology; D013601:T-Lymphocytes / Q000032:analysis
D007136:Immunoglobulins