eMedicine Specialties > Hematology > Red Blood Cells and Disorders

Hemoglobin C Disease: Treatment & Medication

Author: Suzanne M Carter, MS, Senior Genetic Counselor, Associate, Department of Obstetrics and Gynecology, Division of Reproductive Genetics, Montefiore Medical Center, Albert Einstein College of Medicine
Coauthor(s): Susan J Gross, MD, FRCS(C), FACOG, FACMG, Codirector, Division of Reproduction Genetics, Associate Professor, Department of Obstetrics and Gynecology, Albert Einstein College of Medicine
Contributor Information and Disclosures

Updated: Feb 7, 2008

Treatment

Medical Care

  • Patients can usually be evaluated in an outpatient setting.
  • Perform regular eye screening along with fluorescein angiography to assess for neovascularization.

Surgical Care

  • Splenectomy may be indicated, and gallstones may require cholecystectomy.
  • Neovascularization present at the equatorial region of the eye can be treated with a laser to prevent bleeding.

Consultations

  • Dentist
  • Geneticist
  • Hematologist
  • Ophthalmologist

Diet

No special diet is required.

Activity

Physical activities are not restricted.

Medication

Patients with hemoglobin C disease require treatment only for acute problems. Long-term antibiotic prophylaxis is not indicated.

Mineral supplementations

Iron supplementation is used to correct iron deficiency.


Ferrous sulfate (Feosol, Fer-Iron, Ferospace, Slow FE)

A nutritionally essential inorganic substance that allows transportation of oxygen via hemoglobin. Used for simple iron deficiency and iron-deficiency anemia.

Adult

325 mg/d PO

Pediatric

<12 years: Not established
>12 years: Administer as in adults

Absorption enhanced by ascorbic acid; interferes with tetracycline absorption; food and antacids impair absorption

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

GI upset; iron toxicity is observed with ingestion of large amounts and can be fatal, especially in children; parenteral (IV) administration may cause several reactions, including headaches, malaise, fever, generalized lymphadenopathy, arthralgia, and urticaria; can cause severe anaphylaxis; possible phlebitis at infusion site

More on Hemoglobin C Disease

Overview: Hemoglobin C Disease
Differential Diagnoses & Workup: Hemoglobin C Disease
Treatment & Medication: Hemoglobin C Disease
Follow-up: Hemoglobin C Disease
Multimedia: Hemoglobin C Disease
References

References

  1. Modiano D, Luoni G, Sirima BS, et al. Haemoglobin C protects against clinical Plasmodium falciparum malaria. Nature. Nov 15 2001;414(6861):305-8. [Medline].

  2. Rihet P, Flori L, Tall F. Hemoglobin C is associated with reduced Plasmodium falciparum parasitemia and low risk of mild malaria attack. Hum Mol Genet. Jan 1 2004;13(1):1-6.

  3. Olson JF, Ware RE, Schultz WH, Kinney TR. Hemoglobin C disease in infancy and childhood. J Pediatr. Nov 1994;125(5 Pt 1):745-7. [Medline].

  4. Hingorani M, Bentley CR, Jackson H, et al. Retinopathy in haemoglobin C trait. Eye. 1996;10 ( Pt 3):338-42. [Medline].

  5. McBrayer GM, Semes L, Stephens GG. Angioid streaks and AC hemoglobinopathy--a newly discovered association. J Am Optom Assoc. Apr 1993;64(4):250-3. [Medline].

  6. Dare FO, Makinde OO, Faasuba OB. The obstetric performance of sickle cell disease patients and homozygous hemoglobin C disease patients in Ile-Ife, Nigeria. Int J Gynaecol Obstet. Mar 1992;37(3):163-8. [Medline].

  7. Fabry ME, Kaul DK, Raventos C, et al. Some aspects of the pathophysiology of homozygous Hb CC erythrocytes. J Clin Invest. May 1981;67(5):1284-91. [Medline].

  8. Fairhurst RM, Casella JF. Images in clinical medicine. Homozygous hemoglobin C disease. N Engl J Med. Jun 24 2004;350(26):e24. [Medline].

  9. Fairhurst RM, Fujioka H, Hayton K, et al. Aberrant development of Plasmodium falciparum in hemoglobin CC red cells: implications for the malaria protective effect of the homozygous state. Blood. Apr 15 2003;101(8):3309-15. [Medline].

  10. Fort JA, Graham-Pole JR, Chopik J. Vasoocclusion with homozygous hemoglobin-C disease. Am J Pediatr Hematol Oncol. 1988;10(4):323-5. [Medline].

  11. Wickramasinghe SN, Akinyanju OO, Hughes M. Dyserythropoiesis in homozygous haemoglobin C disease. Clin Lab Haematol. 1982;4(4):373-81. [Medline].

Further Reading

Keywords

HbC disease, mild hemolytic anemia, benign hemoglobinopathies, musculoskeletal pain, joint pain, iron deficiency, iron supplementation, angioid streaks, hemoglobin C, gallstones, pigmented gallstones, malaria, Bruch membrane, retinopathy, cholelithiasis, dental infarction, hemolysis, HbC, splenomegaly

Contributor Information and Disclosures

Author

Suzanne M Carter, MS, Senior Genetic Counselor, Associate, Department of Obstetrics and Gynecology, Division of Reproductive Genetics, Montefiore Medical Center, Albert Einstein College of Medicine
Suzanne M Carter, MS is a member of the following medical societies: American Bar Association
Disclosure: Nothing to disclose.

Coauthor(s)

Susan J Gross, MD, FRCS(C), FACOG, FACMG, Codirector, Division of Reproduction Genetics, Associate Professor, Department of Obstetrics and Gynecology, Albert Einstein College of Medicine
Susan J Gross, MD, FRCS(C), FACOG, FACMG is a member of the following medical societies: American College of Medical Genetics, American College of Obstetricians and Gynecologists, American Institute of Ultrasound in Medicine, American Medical Association, American Society of Human Genetics, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Ronald A Sacher, MB, BCh, MD, FRCPC, Director of the Hoxworth Blood Center, Professor, Departments of Internal Medicine and Pathology, University of Cincinnati Medical Center
Ronald A Sacher, MB, BCh, MD, FRCPC is a member of the following medical societies: American Society of Hematology
Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

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 Clinical Oncology, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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