Sideroblastic anemia medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
The anemia can be so severe that support with transfusion is required. These patients usually do not respond to erythropoietin therapy. Some cases have been reported that the anemia is reversed or heme level is improved through use of moderate to high doses of pyrodoxine (vitamin B6).
Medical Therapy[1]
Non pharmacologic and general measures
- 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
- Medications are used to control, anemia, morbidity and complications.
- The two groups are
- Vitamins
- Iron chelators
Vitamins
- Folic acid.
- 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
- Preffered regimen:Aminoxin, adults,2.5-10 mg, PO,once daily
- Preffered regimen:Aminoxin, peadiatrics0.1-1.2 mg, I/M,once daily
- 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 Desferal20-40mg/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.