. 2005 Dec 15;1(4):142–160. doi: 10.1186/1710-1492-1-4-142

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.

In all cases it is strongly recommended to obtain optimal control of hemophagocytic syndrome previous to undertaking hematopoietic stem cell transplant.

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