ESTABLISHMENT OF ERYTHROPOIESIS FOLLOWING BONE-MARROW TRANSPLANTATION IN A PATIENT WITH CONGENITAL HYPOPLASTIC ANEMIA (DIAMOND-BLACKFAN SYNDROME)

  • 1 January 1976
    • journal article
    • research article
    • Vol. 48  (4) , 491-498
Abstract
Marrow transplantation was attempted in a 13 yr old boy with congenital hypoplastic anemia who had never responded to corticosteroid therapy. Prior to the transplant, he had received 238 transfusions, at least 12 of which were from his father. He was prepared for grafting with antilymphocyte globulin, procarbazine and total body irradiation (1000 rad). The patient, whose red cells were Group B, then received marrow cells from his Group O, histocompatible, sister. Reticulocytes, Group O erythrocytes and female leukocytes appeared in the peripheral blood. Erythroid precursors were seen in the patient''s marrow for the 1st time in his life, and all lacked fluorescent Y chromosomes. Dividing cells were all female. After initially progressing well, the patient developed interstitial pneumonia and died 55 days after the transplant. The successful erythroid graft suggested that this patient''s failure to produce red blood cells was due to a defective stem cell rather than to a humoral defect, plasma inhibitor, or abnormal marrow microenvironment. Sibling marrow may be engrafted in patients who have received multiple transfusions, even from a parent.

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