Sideroblastic anemia medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Nazia Fuad M.D.
Overview
The medical therapy for sideroblastic anemia include pyridoxine, thiamine and follic acid. For iron overload iron chelators are used.
Medical Therapy
Non pharmacologic and general measures
The following measures are used to treat sideoblastic anemia.[1]
- Avoid alcohol
- Avoid zinc supplements
- Remove toxic agents
- Lead
- Drugs
- With isoniazid, pyridoxine is given to reverse its adverse effect on heme synthesis.
- Blood transfusion
Pharmacologic measures
Vitamins
- Preffered regimen: Adults 0.4mg, PO,once daily
- preffered regimen peadiatric: 1 mg/day IV/PO/IM/SC initially, then 0.1-0.4 mg/day
- Pyridoxine
- Thiamine
- Preffered regimen:adults 50mg,PO,once daily
- Preffered regimen(1) peadiatric 10-25 mg IV/IM qDay
- Preffered regimen(2) peadiatric10-50 mg PO qDay for 2 weeks,
- Then 5-10 mg/day PO for 1 month
Iron chelators
- Used in patietns having iron overload to remove toxic iron from tissues.
- Preffered rgimen(1) adults Desferal 0.5-1g QD ,I/M
- Preffered rgimen(2) adults Desferal 20-40mg/kg/day SC over 8-24 hours with portable pump providing continuous mini-infusion
- Preffered rgimen(1) peadiatric DesferalSC 1-2g (20-40mg/kg/day) SC over 8-24 hours by small portable pump
- Preffered rgimen(2) peadiatric Desferal IV, 40-50mg/kg/day over 8-12 hours for 5-7 days/ week,maximum, < 60mg/kg/day and an IV infusion rate of <15mg/kg/hr)
References
- ↑ Fujiwara T, Harigae H (December 2013). "Pathophysiology and genetic mutations in congenital sideroblastic anemia". Pediatr Int. 55 (6): 675–9. doi:10.1111/ped.12217. PMID 24003969.