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.

• Avoid alcohol
• Avoid zinc supplements
• Remove toxic agents
  • Lead
  • Drugs
    • Pyridoxine prophylaxis with isoniazid is given to reverse its adverse effect on heme synthesis
• Blood transfusion
  • Blood transfusion can treat anemia but carries the risk of iron overload.
• Phlebotomy

Pharmacologic measures

• Medications are used to control, anemia, morbidity and complications.
• The two groups are
  • Vitamins
  • Iron chelators

Vitamins

• Folic acid
  • Preferred regimen: Adults 0.4mg PO once daily
  • Preferred regimen: Pediatric: 1 mg/day IV/PO/IM/SC initially, then 0.1-0.4 mg/day
• Pyridoxine
  • Preferred regimen: Adults: Aminoxin 2.5-10 mg, PO,once daily
  • Preferred regimen: Pediatric: Aminoxin 0.1-1.2 mg I/M once daily
• Thiamine
  • Preferred regimen: Adults: 50mg PO once daily
  • Preferred regimen(1): Pediatric: 10-25 mg IV/IM qDay
  • Preferred regimen(2): Pediatric: 10-50 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.
  • Preferred regimen(1): Adults: Desferal 0.5-1g I/M daily
  • Preferred regimen(2): Adults: Desferal 20-40mg/kg/day SC over 8-24 hours with portable pump providing continuous mini-infusion
  • Preferred regimen(1): Pediatric: Desferal SC 1-2g (20-40mg/kg/day) SC over 8-24 hours by small portable pump
  • Preferred regimen(2): Pediatric: 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)