Table 3 Guidelines of the European Bone Marrow Transplant/European Society for Inborn Errors group for conditioning regimen for hematopoietic stem cell transplant of inherited hemophagocytic syndromes.
From: Pediatric Hemophagocytic Syndromes: A Diagnostic and Therapeutic Challenge
| In all cases it is strongly recommended to obtain optimal control of hemophagocytic syndrome previous to undertaking hematopoietic stem cell transplant. |
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| A - Genotypical identical donor |
| • Rabbit ATG day-14 to -10 (5 days), dose 10 mg/kg/day |
| • Busulfan: day -10 to -7; age < 6y 5 mg/kg/day × 4;age > 6y 4 mg/kg/day × 4 |
| • Cyclophosphamide: day -5 to -2; 50 mg/kg/day × 4 |
| B - Matched unrelated donor/Phenotype identical related donor/HLA-nonidentical haplo or mismatched family donor. |
| • Rabbit ATG day-14 to -10 (5 days), dose 10 mg/kg |
| • Busulfan: day -10 to -7; age < 6y 5 mg/kg/day × 4;age > 6y 4 mg/kg/day × 4 |
| • Cyclophosphamide: day -5 to -2; 50 mg/kg/day × 4 |
| • Positive selection of CD34+ cells in Stem cell source aiming to have T cell counts <104/kg |
| • Ciclosporine A if T cell counts above 104/kg despite positive selection of CD34+ cells |