Table 2.
Guidelines of the European BoneMarrow Transplant/European Society for Inborn Errors group for the treatment of inherited hemophagocytic syndromes.
| 1 - Intra-venous Methylprednisolone: | |
| • day 1 to day 2 (48 hours) 5 mg/kg/d divided in 2 doses | |
| • day 3 to day 4 (48 hours) 3 mg/kg/d divided in 2 doses | |
| • day 4 2 mg/kg/d till disease control and serum levels of ciclosporine A of ~200-300 ng/ml, then taper to full stop previous to stem cell transplantation. | |
| 2 - Rabbit anti-thymocyte globulins (ATG) | |
| • Day1 to day 5 (5 days) 10 mg/kg/d IV infusion over 6-12 hours | |
| 3 - Cyclosporine A | |
| • begin 48-72 hours after initiating ATG | |
| • 3 mg/kg/d in continuous or in 1 hour BID infusion | |
| • aim is to have serum levels within 200-300 ng/ml | |
| • give oral treatment whenever disease is controlled and maintain until the beginning of the conditioning regimen for stem cell transplantation. | |
| 4 - Intrathecal Methotrexate (MTX) | |
| Doses: as per acute lymphoblastic leukemia protocols, | |
| 6 mg | age 0-1 year |
| 8 mg | age 1-2 years |
| 10 mg | age 2-3 years |
| 12 mg | age >3 years |
| Regimen: DO NOT EXCEED 8 IT treatments | |
| Presence of Central Nervous system involvement on imaging or lumbar tap | |
| 2 LPs/week for 2 weeks | |
| 1 LP/week optimally up to Stem Cell Transplant | |
| Absence of Central Nervous Involvement at diagnosis | |
| 1 LP/week optimally up to Stem Cell Transplant | |