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Fatal diffuse capillaritis after hematopoietic stem-cell transplantation for dyskeratosis congenita despite low-intensity conditioning regimen

Bone Marrow Transplantation volume 36, pages 1103–1105 (2005)Cite this article

In the May 2003 issue of this journal, Dror et al1 reported two children with dyskeratosis congenita (DC) who successfully underwent hematopoietic stem-cell transplantation (HSCT) after low-intensity conditioning. The fludarabine-based conditioning regimen was chosen to avoid the complications described after HSCT for DC, particularly lethal pulmonary and endothelial problems.2, 3, 4, 5 The patients successfully engrafted without severe early or late side effects. Two other successful transplants after low-intensity fludarabine-based conditioning regimens have been reported.6, 7 We thus used the regimen described by Dror et al for a 15-year-old boy with DC. Symptoms of DC were esophageal stenosis with dysphagia, nail dystrophy, reticulated hyperpigmentation, anemia and thrombocytopenia with myelodysplasia. Chromosome breakage studies excluded Fanconi anemia. At the time of transplantation, the patient already had received 62 packed red cell transfusions and 37 platelet transfusions.

Conditioning included fludarabine 30 mg/m2/day i.v. from day −10 to day −5 (total dose 180 mg/m2), cyclophosphamide 60 mg/kg/day i.v. on days −6 and −5 (total dose 120 mg/kg) and equine antithymocyte globulin (ATGAM, Upjohn, Kalamazoo, MI, USA) 40 mg/kg/day from day −4 to −1 (total dose 160 mg/kg). The patient received a non-T-cell-depleted unrelated bone marrow graft, matched for HLA loci A and B at the serological level, and for the DRB1 locus at high-resolution molecular level. The cell dose was 3.8 × 108 nucleated cells/kg. For graft-versus-host disease (GVHD) prophylaxis, the patient was planned to receive i.v. cyclosporine twice daily starting on day −1, targeting a whole blood trough level of 300–400 ng/ml, methotrexate 15 mg/m2 i.v. on day +1 and 10 mg/m2 i.v. on days +3 and +6, and methylprednisolone 0.4 mg/kg/dose twice daily from day –4 to day +28 with subsequent dosage reduction. He received granulocyte-colony-stimulating factor (G-CSF) 5 μg/kg/day s.c. from day +5 until neutrophil counts were >1.0 × 109/l for at least two consecutive days. He also received intravenous immunoglobulin 0.5 g/kg weekly, trimethoprim-sulfamethoxazole 75 mg trimethoprim/m2 twice daily orally starting 1 day before conditioning until day –2, fluconazole 5 mg/kg/day orally from day −3 and acyclovir 250 mg/m2 twice daily i.v. from day –3 to +20. Donor serology for cytomegalovirus was positive, whereas patient serology was negative.

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  1. Service d'Hématologie-Oncologie, Hopital Sainte-Justine, Montreal, Canada

    P Brazzola, M Duval, J C Fournet, F Gauvin, J H Dalle, J Champagne & M A Champagne

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Brazzola, P., Duval, M., Fournet, J. et al. Fatal diffuse capillaritis after hematopoietic stem-cell transplantation for dyskeratosis congenita despite low-intensity conditioning regimen. Bone Marrow Transplant 36, 1103–1105 (2005). https://doi.org/10.1038/sj.bmt.1705171

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