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Safety and efficacy of high dose intravenous desferrioxamine for reduction of iron overload in sickle cell disease
Author(s) -
Kalpatthi Ram,
Peters Brittany,
Kane Ian,
Holloman David,
Rackoff Elizabeth,
Disco Deborah,
Jackson Sherron,
Laver Joseph H,
Abboud Miguel R.
Publication year - 2010
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.22660
Subject(s) - medicine , toxicity , regimen , deferasirox , deferoxamine , gastroenterology , surgery , thalassemia
Background Patients with sickle cell disease (SCD) receiving chronic blood transfusions are at risk of developing iron overload and organ toxicity. Chelation therapy with either subcutaneous (SQ) desferrioxamine (DFO) or oral deferasirox is effective in preventing and reducing iron overload but poses significant challenges with patient compliance. Intravenous (IV) infusions of high dose DFO have been utilized in non‐compliant patients with heavy iron overload in small case series. Procedure We review our experience of high dose IV DFO in 27 patients with SCD who had significant iron overload and were noncompliant with subcutaneous (SQ) DFO. All patients were treated in‐hospital with DFO 15 mg/kg/hr IV for 48 hr every 2–4 weeks with a mean duration of 19.6 months. Results We observed a significant decrease in liver iron burden with high dose intermittent IV DFO. Histological examination of liver biopsies revealed a decrease in the grade of liver iron storage. Also there was significant improvement in liver enzymes (ALT, AST) after high dose IV DFO. No audiologic or ophthalmologic toxicity or acute or chronic pulmonary complications were observed. Conclusions In our cohort of patients with SCD we observed a significant decrease in liver iron burden with high dose IV DFO. Our patients tolerated the therapy well without any major toxicity. This regimen is safe and may be an option for poorly compliant patients with significant iron overload. Pediatr Blood Cancer. 2010;55:1338–1342. © 2010 Wiley‐Liss, Inc.