Sideroblastic anemia medical therapy

Jump to: navigation, search


Sideroblastic anemia Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Sideroblastic Anemia from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X Ray

CT

MRI

Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Sideroblastic anemia medical therapy On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Sideroblastic anemia medical therapy

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Sideroblastic anemia medical therapy

CDC on Sideroblastic anemia medical therapy

Sideroblastic anemia medical therapy in the news

Blogs on Sideroblastic anemia medical therapy

Directions to Hospitals Treating Sideroblastic anemia

Risk calculators and risk factors for Sideroblastic anemia medical therapy

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][2]

  • Avoid alcohol
  • Avoid zinc supplements
  • Remove toxic agents
  • Blood transfusion
    • Blood transfusion can treat anemia but carries the risk of iron overload.
  • Phlebotomy

Pharmacologic measures

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)

References

  1. 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.
  2. 2.0 2.1 Mason DY, Emerson PM (February 1973). "Primary acquired sideroblastic anaemia: response to treatment with pyridoxal-5-phosphate". Br Med J. 1 (5850): 389–90. PMC 1588335. PMID 4691061.

Linked-in.jpg