Table 2 Guidelines of the European BoneMarrow Transplant/European Society for Inborn Errors group for the treatment of inherited hemophagocytic syndromes.

   • 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

   8 mg

   10 mg

   12 mg

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