Hematopoietic Chimerism After BMT for Sickle Cell Anemia
Stable Mixed Hematopoietic Chimerism After Bone
Marrow Transplantation for Sickle Cell Anemia
M.C. Walters, M. Patience, W. Leisenring, Z.R. Rogers, V.M. Aquino, G.R. Buchanan,
I.A.G. Roberts, A.M. Yeager, L. Hsu, T. Adamkiewicz, J. Kurtzberg, E. Vichinsky, B. Storer,
R. Storb, K.M. Sullivan
Biology of Blood and Marrow Transplantation 7:665-673 (2001)
© 2001 American Society for Blood and Marrow Transplantation
ABSTRACT:
A multicenter investigation of allogeneic bone marrow transplantation for children with sickle cell disease was conducted
that included 27 European and North American transplant centers. Fifty-nine patients who ranged in age
from 3.3 to 15.9 years (median, 10.1 years) received HLA-identical sibling marrow allografts between September
1991 and April 2000. Fifty-five patients survive, and 50 survive free from sickle cell disease, with a median follow-up
of 42.2 months (range, 11.8 to 115 months) after transplantation. Of the 50 patients with successful allografts, 13
developed stable mixed donor-host hematopoietic chimerism. The level of donor chimerism, measured >=6 months
after transplantation in peripheral blood, varied between 90% and 99% in 8 patients. Five additional patients had a
lower proportion of donor cells (range, 11% to 74%). Among these 5 patients, hemoglobin levels varied between
11.2 and 14.2 g/dL (median, 11.3 g/dL; mean, 12.0 g/dL). In patients who had donors with a normal hemoglobin
genotype (Hb), the Hb S fractions were 0%, 0%, and 7%, corresponding to donor chimerism levels of 67%, 74%,
and 11%, respectively. Among patients who had donors with sickle trait, the Hb S fractions were 36% and 37%, corresponding
to donor chimerism levels of 25% and 60%, respectively. Thus, allograft recipients with stable mixed
chimerism had Hb S levels similar to donor levels, and only 1 patient required a red blood cell transfusion beyond
90 days posttransplantation. None of the patients have experienced painful events or other clinical complications
related to sickle cell disease after transplantation. These observations strongly suggest that patients with sickle cell
disease who develop persistent mixed hematopoietic chimerism after transplantation experience a significant ameliorative
effect.
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