Sickle cell anemia
Revision as of 18:32, 15 March 2018 by Warner-admin (talk | contribs) (Text replacement - "'''Use of this site is subject to you reading and agreeing with the terms set forth in the disclaimer. If this is your first time visiting, we suggest you read the tutorial.'''" to "<!--'''Use of this site is subject to you reading and agreeing with the terms set forth in the disclaimer. If this is your first time visiting, we suggest you read the tutorial.'''-->")
Is there a regimen missing from this list? Would you like to share a different dosage/schedule or an additional reference for a regimen? Have you noticed an error? Do you have an idea that will help the site grow to better meet your needs and the needs of many others? You are invited to contribute to the site.
| Section editors | |||
|---|---|---|---|
 |
Shruti Chaturvedi, MBBS, MSCI Baltimore, MD |
 |
Benjamin Tillman, MD Nashville, TN |
6 regimens on this page
6 variants on this page
|
Guidelines
NHLBI
All lines of therapy
Hydroxyurea monotherapy
| back to top |
Regimen
| Study | Evidence | Comparator | Efficacy |
|---|---|---|---|
| Charache et al. 1995 (MSH) | Phase III | Placebo | Superior annual rate of crises |
Chemotherapy
- Hydroxyurea (Hydrea) 15 mg/kg/day PO
Doses were subsequently adjusted, see article for details.
References
- Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SV, McMahon RP, Bonds DR. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the multicenter Study of Hydroxyurea in Sickle Cell Anemia. N Engl J Med. 1995 May 18;332(20):1317-22. link to original article contains protocol PubMed
- Update: Steinberg MH, Barton F, Castro O, Pegelow CH, Ballas SK, Kutlar A, Orringer E, Bellevue R, Olivieri N, Eckman J, Varma M, Ramirez G, Adler B, Smith W, Carlos T, Ataga K, DeCastro L, Bigelow C, Saunthararajah Y, Telfer M, Vichinsky E, Claster S, Shurin S, Bridges K, Waclawiw M, Bonds D, Terrin M. Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment. JAMA. 2003 Apr 2;289(13):1645-51. Erratum in: JAMA. 2003 Aug 13;290(6):756. link to original article PubMed
- QoL analysis: Ballas SK, Barton FB, Waclawiw MA, Swerdlow P, Eckman JR, Pegelow CH, Koshy M, Barton BA, Bonds DR. Hydroxyurea and sickle cell anemia: effect on quality of life. Health Qual Life Outcomes. 2006 Aug 31;4:59. link to original article link to PMC article PubMed
L-glutamine monotherapy
| back to top |
Regimen
| Study | Evidence | Comparator | Efficacy |
|---|---|---|---|
| No publication | Phase III | Placebo | Superior reduction in the number of sickle cell crises through week 48 |
Note: this trial is not available in published form; instructions here are from the ClinicalTrials.gov page.
Therapy
- L-glutamine (Endari) 0.3 g/kg PO BID
48-week course
References
RBC transfusions
| back to top |
Regimen
| Study | Evidence | Comparator | Efficacy |
|---|---|---|---|
| DeBaun et al. 2014 | Phase III | Observation | Seems to have fewer cerebral infarcts |
To be completed
References
- DeBaun MR, Gordon M, McKinstry RC, Noetzel MJ, White DA, Sarnaik SA, Meier ER, Howard TH, Majumdar S, Inusa BP, Telfer PT, Kirby-Allen M, McCavit TL, Kamdem A, Airewele G, Woods GM, Berman B, Panepinto JA, Fuh BR, Kwiatkowski JL, King AA, Fixler JM, Rhodes MM, Thompson AA, Heiny ME, Redding-Lallinger RC, Kirkham FJ, Dixon N, Gonzalez CE, Kalinyak KA, Quinn CT, Strouse JJ, Miller JP, Lehmann H, Kraut MA, Ball WS Jr, Hirtz D, Casella JF. Controlled trial of transfusions for silent cerebral infarcts in sickle cell anemia. N Engl J Med. 2014 Aug 21;371(8):699-710. link to original article link to PMC article PubMed