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Chédiak–Higashi syndrome: hematopoietic chimerism corrects genetic defect

Bone Marrow Transplantation volume 27, pages 1211–1213 (2001)Cite this article

Abstract

Chédiak–Higashi syndrome is a rare autosomal recessive disorder, primarily affecting neutrophils, and is often lethal by the third decade of life. Bone marrow transplantation is the only curative therapy currently available. This case describes a child undergoing a bone marrow transplant from a matched sibling donor, resulting in hematopoietic chimerism with only a small percentage of donor neutrophils found long term. The presence of a small percentage of donor neutrophils has resulted in normal development and no increased incidence of infections. Hematopoietic chimerism offers a cure with a potential reduction in the side-effects that result from marrow transplantation and the associated preparative therapies. Bone Marrow Transplantation (2001) 27, 1211–1213.

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Authors and Affiliations

  1. Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA, USA

    ME Trigg

  2. Department of Pediatrics, Division of Blood and Bone Marrow Transplantation, AI duPont Hospital for Children, Wilmington, DE, USA

    ME Trigg & R Schugar

Authors
  1. ME Trigg
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  2. R Schugar
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Trigg, M., Schugar, R. Chédiak–Higashi syndrome: hematopoietic chimerism corrects genetic defect. Bone Marrow Transplant 27, 1211–1213 (2001). https://doi.org/10.1038/sj.bmt.1703058

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