eMedicine Specialties > Hematology > Red Blood Cells and Disorders

Thalassemia, Alpha: Treatment & Medication

Author: Samer A Bleibel, MD, Staff Physician, Department of Internal Medicine, Wayne State University, St John's Hospital and Medical Centers
Coauthor(s): Robert J Leonard, MD, Clinical Assistant Professor, Department of Medicine, Wayne State University School of Medicine; Jennifer L Jones-Crawford, MD, Fellow, Department of Hematology/Oncology, Medical College of Georgia; Abdullah Kutlar, MD, Director of Sickle Cell Center, Fellowship Program Director, Professor, Department of Internal Medicine, Section of Hematology and Oncology, Medical College of Georgia; Linda K Hendricks, MD, Assistant Professor, Department of Internal Medicine, Section of Hematology and Oncology, Mercer University School of Medicine
Contributor Information and Disclosures

Updated: Aug 26, 2009

Treatment

Medical Care

  • Avoid iron supplementation as it contributes to iron overload and does not affect hematologic values or cell morphology.
  • Administer folate supplementation to provide adequate amounts of the vitamin for increased utilization resulting from the hemolytic process and high bone marrow turnover rate.
  • Provide prompt attention to infection, especially in children who have had splenectomy, and administer appropriate vaccines to these individuals.
  • Administer blood transfusions only if necessary.
  • If chronic transfusion is needed (hemoglobin H disease), iron chelation therapy should be considered to avoid iron overloading.

Surgical Care

  • Hemoglobin H disease
    • Perform splenectomy if transfusion requirements are increasing.
    • Surgical or orthodontic correction may be necessary to correct skeletal deformities of the skull and maxilla caused by erythroid hyperplasia.

More on Thalassemia, Alpha

Overview: Thalassemia, Alpha
Differential Diagnoses & Workup: Thalassemia, Alpha
Treatment & Medication: Thalassemia, Alpha
Follow-up: Thalassemia, Alpha
Multimedia: Thalassemia, Alpha
References
Further Reading
[ CLOSE WINDOW ]

References

  1. Muncie HL Jr, Campbell J. Alpha and beta thalassemia. Am Fam Physician. Aug 15 2009;80(4):339-44. [Medline].

  2. Higgs DR, Weatherall DJ. The alpha thalassaemias. Cell Mol Life Sci. Apr 2009;66(7):1154-62. [Medline].

  3. Singer ST. Variable clinical phenotypes of alpha-thalassemia syndromes. ScientificWorldJournal. Jul 13 2009;9:615-25. [Medline].

  4. Haas PS, Roy NB, Gibbons RJ, Deville MA, Fisher C, Schwabe M, et al. The role of X-inactivation in the gender bias of patients with acquired alpha-thalassaemia and myelodysplastic syndrome (ATMDS). Br J Haematol. Feb 2009;144(4):538-45. [Medline].

  5. Giambona A, Passarello C, Renda D, Maggio A. The significance of the hemoglobin A(2) value in screening for hemoglobinopathies. Clin Biochem. Jul 8 2009;[Medline].

  6. Liu J, Tang N, Liu Q, Wang L, Han H, Cai R, et al. Improvement in the Detection of alpha0- and Deletional alpha-Thalassemia by Real-Time PCR Combined with Dissociation Curve Analysis. Acta Haematol. Aug 15 2009;122(1):17-22. [Medline].

  7. Chen YL, Shih CJ, Ferrance J, Chang YS, Chang JG, Wu SM. Genotyping of alpha-thalassemia deletions using multiplex polymerase chain reactions and gold nanoparticle-filled capillary electrophoresis. J Chromatogr A. Feb 13 2009;1216(7):1206-12. [Medline].

  8. Ribeiro DM, Sonati MF. Regulation of human alpha-globin gene expression and alpha-thalassemia. Genet Mol Res. Oct 14 2008;7(4):1045-53. [Medline].

  9. Arcasoy MO, Gallagher PG. Hematologic disorders and nonimmune hydrops fetalis. Semin Perinatol. Dec 1995;19(6):502-15. [Medline].

  10. Benz EJ. Clinical Manifestations of the thalassemias. UpToDate. 2006.

  11. Bernini LF, Harteveld CL. Alpha-thalassaemia. Baillieres Clin Haematol. Mar 1998;11(1):53-90. [Medline].

  12. Bunn HF, Forget BG. Hemoglobin. In: Molecular, Genetic and Clinical Aspects. Philadelphia, Pa: WB Saunders; 1986.

  13. Chui DH, Fucharoen S, Chan V. Hemoglobin H disease: not necessarily a benign disorder. Blood. Feb 1 2003;101(3):791-800. [Medline].

  14. Chui DH, Waye JS. Hydrops fetalis caused by alpha-thalassemia: an emerging health care problem. Blood. Apr 1 1998;91(7):2213-22. [Medline].

  15. Clegg JB, Weatherall DJ. Thalassemia and malaria: new insights into an old problem. Proc Assoc Am Physicians. Jul-Aug 1999;111(4):278-82. [Medline].

  16. Higgs DR. Hamm-Wasserman LectureGene Regulation in Hematopoiesis: New Lessons from Thalassemia. Hematology. 2004.

  17. Kazazian HH Jr, Dowling CE, Hurwitz RL, Coleman M, Stopeck A, Adams JG 3rd. Dominant thalassemia-like phenotypes associated with mutations in exon 3 of the beta-globin gene. Blood. Jun 1 1992;79(11):3014-8. [Medline].

  18. Lee R, Foerster J, Lukens J. The thalassemias and related disorders:. In: quantitative disorders of hemoglobin synthesis. In: Wintrobe's Clinical Hematology. Philadelphia, Pa: Lippincott, Williams, and Wilkins; 1999:1405-1448.

  19. Schell T, Kulozik AE, Hentze MW. Integration of splicing, transport and translation to achieve mRNA quality control by the nonsense-mediated decay pathway. Genome Biol. 2002;3(3):REVIEWS1006. [Medline].

  20. Schrier SL. Pathophysiology of alpha thalassemia. UpToDate. 2006.

  21. Schrier SL. Thalassemia: pathophysiology of red cell changes. Annu Rev Med. 1994;45:211-8. [Medline].

  22. Sgourou A, Routledge S, Antoniou M, Papachatzopoulou A, Psiouri L, Athanassiadou A. Thalassaemia mutations within the 5'UTR of the human beta-globin gene disrupt transcription. Br J Haematol. Mar 2004;124(6):828-35. [Medline].

  23. Steensma DP, Gibbons RJ, Higgs DR. Acquired alpha-thalassemia in association with myelodysplastic syndrome and other hematologic malignancies. Blood. Jan 15 2005;105(2):443-52. [Medline].

  24. Vichinsky EP, MacKlin EA, Waye JS, Lorey F, Olivieri NF. Changes in the epidemiology of thalassemia in North America: a new minority disease. Pediatrics. Dec 2005;116(6):e818-25. [Medline].

  25. Weatherall D. The molecular basis for phenotypic variability of the common thalassaemias. Mol Med Today. Apr 1995;1(1):15-20. [Medline].

  26. Weatherall DJ, Clegg JB. The Thalassaemia Syndromes Fourth Edition. 2004.

  27. Weatherall, DJ, Clegg, JB, Higgs, DR, et al. The hemoglobinopathies. In:. In: Scriver, CR, Beaudet, AL, Sly, WS, Valle, D (Eds). The Metabolic and Molecular Bases of Inherited Disease. 7th edition. New York: McGraw-Hill; 1995:p.3417.

[ CLOSE WINDOW ]

Further Reading

Related eMedicine Topics

Clinical Trials
 National Guideline Clearinghouse

[ CLOSE WINDOW ]

Keywords

alpha thalassemia, hemoglobin H disease, hydrops fetalis, alpha thalassemia minor, alpha thalassemia major, thalassemia intermedia, alpha globin chains, beta thalassemia, beta globin chains, silent carrier state, homozygous alpha+ thalassemia, heterozygous alpha0 thalassemia, hemoglobin H, hemoglobin Bart, hemoglobin Bart’s

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Samer A Bleibel, MD, Staff Physician, Department of Internal Medicine, Wayne State University, St John's Hospital and Medical Centers
Samer A Bleibel, MD is a member of the following medical societies: American College of Physicians
Disclosure: Nothing to disclose.

Coauthor(s)

Robert J Leonard, MD, Clinical Assistant Professor, Department of Medicine, Wayne State University School of Medicine
Disclosure: Nothing to disclose.

Jennifer L Jones-Crawford, MD, Fellow, Department of Hematology/Oncology, Medical College of Georgia
Jennifer L Jones-Crawford, MD is a member of the following medical societies: American College of Physicians and American Society of Hematology
Disclosure: Nothing to disclose.

Abdullah Kutlar, MD, Director of Sickle Cell Center, Fellowship Program Director, Professor, Department of Internal Medicine, Section of Hematology and Oncology, Medical College of Georgia
Abdullah Kutlar, MD is a member of the following medical societies: American Society of Hematology
Disclosure: Nothing to disclose.

Linda K Hendricks, MD, Assistant Professor, Department of Internal Medicine, Section of Hematology and Oncology, Mercer University School of Medicine
Linda K Hendricks, MD is a member of the following medical societies: American Society of Hematology
Disclosure: Nothing to disclose.

Medical Editor

Wadie F Bahou, MD, Chief, Division of Hematology, Hematology/Oncology Fellowship Director, Professor, Department of Internal Medicine, State University of New York at Stony Brook
Wadie F Bahou, MD is a member of the following medical societies: American Society of Hematology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Marcel E Conrad, MD, (Retired) Distinguished Professor of Medicine, University of South Alabama
Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, and Southwest Oncology Group
Disclosure: No financial interests None None

CME Editor

Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.