Sideroblastic anemia medical therapy: Difference between revisions
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Created page with "__NOTOC__ {{Sideroblastic anemia}} {{CMG}} ==Overview== The anemia can be so severe that support with transfusion is required. These patients usually do not respond to [[eryth..." |
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__NOTOC__ | __NOTOC__ | ||
{{Sideroblastic anemia}} | {{Sideroblastic anemia}} | ||
{{CMG}} | {{CMG}} {{AE}} {{N.F}} | ||
==Overview== | |||
The anemia | == Overview == | ||
The medical therapy for sideroblastic anemia include [[pyridoxine]], [[thiamine]] and follic acid. For [[iron]] overload iron [[Chelation|chelators]] are used. | |||
==Medical Therapy== | |||
=== Non pharmacologic and general measures === | |||
The following measures are used to treat sideoblastic anemia.<ref name="pmid24003969">{{cite journal |vauthors=Fujiwara T, Harigae H |title=Pathophysiology and genetic mutations in congenital sideroblastic anemia |journal=Pediatr Int |volume=55 |issue=6 |pages=675–9 |date=December 2013 |pmid=24003969 |doi=10.1111/ped.12217 |url=}}</ref><ref name="pmid4691061">{{cite journal |vauthors=Mason DY, Emerson PM |title=Primary acquired sideroblastic anaemia: response to treatment with pyridoxal-5-phosphate |journal=Br Med J |volume=1 |issue=5850 |pages=389–90 |date=February 1973 |pmid=4691061 |pmc=1588335 |doi= |url=}}</ref> | |||
* 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.<ref name="pmid4691061" /> | |||
* 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) | |||
==References== | ==References== | ||
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{{WS}} | {{WS}} | ||
[[Category:Hematology]] | [[Category:Hematology]] | ||
Latest revision as of 19:58, 19 December 2018
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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][2]
- 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
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
- 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
- ↑ 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.
- ↑ 2.0 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.