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In patients with Chediak–Higashi syndrome undergoing allogeneic SCT, does adding etoposide to the conditioning regimen improve the outcome?

Bone Marrow Transplantation volume 40, page 603 (2007)Cite this article

Chediak–Higashi syndrome (CHS) is invariably fatal if left untreated; patients usually die during the accelerated phase, which in many ways resembles familial hemophagocytic lymphohistiocytosis (FHLH). Eapen et al.1 recently published the results of a multi-institutional study of 35 patients with CHS who underwent allogeneic stem cell transplantation (SCT); they reported an overall survival rate of 62%. This survival rate is comparable to that reported in patients with FHLH post-allogeneic SCT as shown recently by Ouachée-Chardin et al.2 in a group of 58 FHLH patients, where the patients received etoposide in addition to busulfan and cyclophosphamide in the preparative regimen; the overall survival rate was 58.5%.

In the study by Eapen et al.,1 the combination of busulfan and cyclophosphamide was the most common preparative regimen, but some patients received etoposide in addition; the authors correlated the survival and disease status post-SCT with some pre-transplant factors such as disease status pre-transplant and donor type, but they did not address the impact of the conditioning regimen (namely with or without etoposide) on the outcome, although on the other hand the small number of patients in each group (with or without etoposide) in that study may not have allowed such an analysis.

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References

  1. Eapen M, Delaat CA, Baker KS, Cairo MS, Cowan MJ, Kurtzberg J et al. Hematopoietic cell transplantation for Chediak–Higashi syndrome. Bone Marrow Transplant 2007; 39: 411–415.

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Authors and Affiliations

  1. Department of Pediatric Hematology–Oncology, Section of Pediatric Stem Cell Transplantation, King Faisal Specialist Hospital and Research Center (KFSHRC), Riyadh, Saudi Arabia

    M Ayas

  2. Department of Pediatrics, Section of Allergy and Immunology, King Faisal Specialist Hospital and Research Center (KFSHRC), Riyadh, Saudi Arabia

    A Al-Ghonaium

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  1. M Ayas
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  2. A Al-Ghonaium
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Correspondence to M Ayas.

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Ayas, M., Al-Ghonaium, A. In patients with Chediak–Higashi syndrome undergoing allogeneic SCT, does adding etoposide to the conditioning regimen improve the outcome?. Bone Marrow Transplant 40, 603 (2007). https://doi.org/10.1038/sj.bmt.1705774

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