Abstract
Bone marrow transplantation (BMT) has occasionally been complicated by the development of hemolytic anemia from specific alloantibodies to either ABO or Rh antigens. We report a case of post-BMT hemolytic anemia in severe combined immunodeficiency (SCID) due to IgG antibodies that agglutinate all red blood cells (RBC) in a broad panel of allotypes.
A 15 month old female with recurrent respiratory distress and oral candidiasis had panhypogammaglobulinemia, decreased T-lymphooytes (312/mm3) and mitogen responsiveness. A diagnosis of SCID prompted transplantation of unfractionated bone marrow from her HLA identical, MLC non-reactive mother without prior ablative therapy or post-transplant graft-vs-host disease (GvHD) prophylaxis. Bone marrow engraftment proceeded rapidly without evidence of GvHD. Eight weeks after BMT her hemoglobin acutely fell to 3.4g/dl with 42% reticulocytes. Direct and indirect antiglobulin tests were positive. Although the donor (A−) and recipient (A+) differed at two Rh loci, the antibody specificity was not restricted to the Rh allotypes.
This appears to be the first report of post-BMT immune hemolytic anemia in SCID due to an antibody of broad rather than allospecificity. Ongoing studies may provide a better understanding of the mechanisms of autoantibody production in immune hemolytic anemia.
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Foster, D., Mamlok, R., Ozkaragoz, F. et al. IMMUNE HEMOLYTIC ANEMIA AFTER BONE MARROW TRANSPLANTATION FOR SEVERE COMBINED IMMUNODEFICIENCY. Pediatr Res 21 (Suppl 4), 311 (1987). https://doi.org/10.1203/00006450-198704010-00866
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DOI: https://doi.org/10.1203/00006450-198704010-00866
