Sideroblastic anemia
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| Sideroblastic anemia Classification and external resources | |
 | |
|---|---|
| Sideroblastic (microcytic) anemia[1] | |
| ICD-10 | D64.0-D64.3 |
| ICD-9 | 285.0 |
| DiseasesDB | 12110 |
| MeSH | D000756 |
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Sideroblastic anemia is caused by the abnormal production of red blood cells as part of myelodysplastic syndrome, which can evolve into hematological malignancies (especially acute myelogenous leukemia). Thus, the body has iron available, but cannot incorporate it into hemoglobin.
Causes
The common feature of these causes is a failure to completely form heme - whose biosynthesis takes place partly in the mitochondrion. This leads to deposits of iron in the mitochondria that form a ring around the nucleus of the developing red blood cell. Sometimes the disorder represents a stage in evolution of a generalized bone marrow disorder that may ultimately terminate in acute leukemia.
- Toxins: lead or zinc poisoning
- Drug-induced: ethanol, isoniazid, chloramphenicol, cycloserine
- Nutritional: pyridoxine or copper deficiency
- Genetic: ALA synthase deficiency (X-linked)
Diagnosis
Ringed sideroblasts are seen in the bone marrow.
Laboratory findings
- Increased ferritin levels
- Decreased total iron-binding capacity
- Hematocrit of about 20-30%
- Serum Iron: High
- High transferrin saturation
- The mean corpuscular volume or MCV is usually normal or slightly increased; although it may occasionally be low, leading to confusion with iron deficiency.
- With lead poisoning, see coarse basophilic stippling of red blood cells on peripheral blood smear
- Specific test: Prussian Blue stain of RBC in marrow. Shows ringed sideroblasts.
Treatment
Occasionally, the anemia is 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.) In severe cases of SBA Bone Marrow Transplant is also an option with limited information about the success rate. Some cases are listed on MedLine and various other medical cites.
See also
References
- ^ "Sideroblastic Anemia" Tierney/McPhee/Papadakis Current Medical Diagnosis and Treatment. 45th Ed. McGraw-Hill. ISBN: 0-07-145410-1.
- ^ "Sideroblastic Anemia" Tierney/McPhee/Papadakis Current Medical Diagnosis and Treatment. 45th Ed. McGraw-Hill. ISBN: 0-07-145410-1.
External links
- Sideroblastic Anemias: Introduction - Information Center for Sickle Cell and Thalassemic Disorders
- A concise description of this group of diseases from the Iron Disorders Institute
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| Evidence Based Medicine Regarding Sideroblastic anemia | Cochrane Collaboration on Sideroblastic anemia • Bandolier on Sideroblastic anemia • TRIP on Sideroblastic anemia |
| Cost Effectiveness of Sideroblastic anemia | Cost Effectiveness of Sideroblastic anemia |
| Clinical Trials Involving Sideroblastic anemia | Ongoing Trials on Sideroblastic anemia at Clinical Trials.gov • Trial results on Sideroblastic anemia • Clinical Trials on Sideroblastic anemia at Google |
| Guidelines / Policies / Government Resources (FDA/CDC) Regarding Sideroblastic anemia | US National Guidelines Clearinghouse on Sideroblastic anemia • NICE Guidance on Sideroblastic anemia • NHS PRODIGY Guidance • FDA on Sideroblastic anemia • CDC on Sideroblastic anemia |
| Textbook Information on Sideroblastic anemia | Books and Textbook Information on Sideroblastic anemia |
| Pharmacology Resources on Sideroblastic anemia | Dosing of Sideroblastic anemia • Drug interactions with Sideroblastic anemia • Side effects of Sideroblastic anemia • Allergic reactions to Sideroblastic anemia • Overdose information on Sideroblastic anemia • Carcinogenicity information on Sideroblastic anemia • Sideroblastic anemia in pregnancy • Pharmacokinetics of Sideroblastic anemia • |
| Genetics, Pharmacogenomics, and Proteinomics of Sideroblastic anemia | Genetics of Sideroblastic anemia • Pharmacogenomics of Sideroblastic anemia • Proteomics of Sideroblastic anemia |
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| Healthcare Provider Resources on Sideroblastic anemia | Symptoms of Sideroblastic anemia • Causes & Risk Factors for Sideroblastic anemia • Diagnostic studies for Sideroblastic anemia • Treatment of Sideroblastic anemia |
| Continuing Medical Education (CME) Programs on Sideroblastic anemia | CME Programs on Sideroblastic anemia |
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Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
