Department of Microbiology and Immunology, Indiana University School of Medicine, Indianapolis, Indiana, USA.
Address correspondence to: Hal E. Broxmeyer, Indiana University School of Medicine, Department of Microbiology and Immunology, 950 West Walnut Street, R2-302, Indianapolis, Indiana 46202-5181, USA. Phone: 317.274.7510; Email: firstname.lastname@example.org.
First published June 25, 2018 - More info
WHIM (warts, hypogammaglobulinemia, infections, and myelokathexis) syndrome is a genetic autoimmune disorder that results from gain-of-function mutations in the gene encoding chemokine receptor CXCR4. A previous study characterized a patient with WHIM who underwent a chromothriptic event that resulted in spontaneous deletion of the WHIM allele in a single hematopoietic stem cell and subsequent cure of the disease. In this issue of the JCI, Gao et al. extend this work and show that Cxcl4-haplosufficient bone marrow has a selective advantage for long-term engraftment in murine WHIM models. Moreover, successful engraftment occurred without prior conditioning of recipients. Together, these results have important implications for improving hematopoietic stem/progenitor cell transplant not only for patients with WHIM but also for all patients who may require the procedure.
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