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Blood, Vol. 114, Issue 1, 211-218, July 2, 2009
Hematopoietic stem cell transplantation in Griscelli syndrome type 2: a single-center report on 10 patients
Blood Pachlopnik Schmid et al.
114: 211
Supplementary materials for: Pachlopnik Schmid et al
Files in this Data Supplement:
- Figure S1. Treatment protocol overview for severe hemophagocytic syndromes (JPG, 205 KB)
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Patients P1, P2, P5, P6, and P7 were treated for active HLH disease with solumedrol (daily administration; initial dose, according to severity, up to 4 mg/kg/d i.v.; followed by a dose reduction as soon as possbile, according to response and severity), cyclosporin A (CSA) (aiming residual level around 150 ng/ml (monoclonal), initial dose 2.5–5.0 mg/kg over 24 h i.v. or 6 mg/kg/d p.o. (divided in 2 doses), if kidney function normal), rabbit anti-thymoglobulin (ATG)(2–10 mg/kg/d i.v., dose according to severity, usually 5 mg/kg/d) and, in case of liquor pleocytosis (lymphocytes and/or monocytes/macrophages), methotrexate (doses by age: <1 year 6 mg, 1–2 years 8 mg, 2–3 years 10 mg, >3 years 12 mg i.t. each dose) combined with hydrocortisone (doses by age: <1 year 6 mg, 1–2 years 8 mg, 2–3 years 10 mg, >3 years 12 mg i.t. each dose). The i.t. treatment was stopped after 4–6 doses or sooner, if cerebrospinal fluid examination was normal. Supportive treatment consisted of i.v. immunoglobulins and cotrimoxazole in all cases, antihistaminics before and solumedrol during ATG infusion in all cases and folic acid if applicable.
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