Relationship between Mixed Donor–Recipient Chimerism and Disease Recurrence after Hematopoietic Cell Transplantation for Sickle Cell Disease
Abraham, Allistair; Hsieh, Matthew; Eapen, Mary; Fitzhugh, Courtney; Carreras, Jeanette; Keesler, Daniel; Guilcher, Gregory; Kamani, Naynesh; Walters, Mark C.; Boelens, Jaap J.; Tisdale, John; Shenoy, Shalini; National Institutes of Health; Center for International Blood and Marrow Transplant Research
(2017) Biology of Blood and Marrow Transplantation, volume 23, issue 12, pp. 2178 - 2183
(Article)
Abstract
Mixed donor chimerism after hematopoietic cell transplantation for sickle cell disease (SCD) can result in resolution of disease symptoms, but symptoms recur when donor chimerism is critically low. The relationship between chimerism, hemoglobin S (HbS) level, and symptomatic disease was correlated retrospectively in 95 patients who had chimerism reports available
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at day 100 and at 1 and 2 years after transplantation. Recurrent disease was defined as recurrence of vaso-occlusive crises, acute chest syndrome, stroke, and/or HbS levels > 50%. Thirty-five patients maintained full donor chimerism (myeloid or whole blood) through 2 years. Donor chimerism was less than 10% (defined as graft failure) in 13 patients during this period. Mixed chimerism was reported in the remaining 47 patients (range, 10% to 94%). The lowest documented donor chimerism without symptomatic disease was 26%. Of 12 surviving patients with recurrent disease, 2 had recurrence of symptoms before documented graft failure (donor chimerism of 11% and 17%, respectively). Three patients underwent second transplantation for graft failure. None received donor leukocyte infusion to maintain mixed chimerism or prevent graft failure. We conclude stable donor chimerism greater than 25% is associated with resolution of SCD-related symptoms, and HbS levels in transplant recipients should be interpreted in context of the sickle trait status of the donors.
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Keywords: Disease symptoms, Mixed chimerism, Sickle cell disease, Transplant, Hematology, Transplantation
ISSN: 1083-8791
Publisher: Elsevier Inc.
Note: Funding Information: Financial disclosure: The CIBMTR is funded by Public Health Service grant U24-CA076518 from the National Cancer Institute, the National Heart, Lung and Blood Institute, and the National Institute of Allergy and Infectious Diseases and a contract with the Health Resources and Services Administration (HHSH250201200016C) and the Intramural Program of the National heart Lung and Blood Institute and the National Institute of Diabetes and Digestive and Kidney diseases, National Institutes of Health. The views expressed in this article do not reflect the official policy or position of the National Institutes of Health, Health Resources and Services Administration, or any other agency of the U.S. Government. Funding Information: Financial disclosure: The CIBMTR is funded by Public Health Service grant U24-CA076518 from the National Cancer Institute , the National Heart, Lung and Blood Institute, and the National Institute of Allergy and Infectious Diseases and a contract with the Health Resources and Services Administration (HHSH250201200016C) and the Intramural Program of the National heart Lung and Blood Institute and the National Institute of Diabetes and Digestive and Kidney diseases, National Institutes of Health. The views expressed in this article do not reflect the official policy or position of the National Institutes of Health, Health Resources and Services Administration, or any other agency of the U.S. Government. Publisher Copyright: © 2017 The American Society for Blood and Marrow Transplantation
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