eMedicine Specialties > Hematology > Red Blood Cells and Disorders

Hemoglobin C Disease: Follow-up

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: Nov 24, 2009

Follow-up

Further Inpatient Care

Admit patients if they require a cholecystectomy or splenectomy.

Further Outpatient Care

Monitor patients for anemia.

Inpatient & Outpatient Medications

  • As with any chronic hemolytic anemia, folic acid at a dose of 1 mg/d PO is indicated.
  • Initiate short-term iron supplementation only if iron stores are low.

Complications

  • Cholelithiasis may occur.
  • Splenomegaly may occur.
  • Angioid streaks may occur.
  • As in all chronic hemolytic anemias, a so-called aplastic crisis may occur, in which erythropoiesis becomes greatly impaired and more severe anemia ensues as a result of the inability of the marrow to produce erythroid cells at the rate required to compensate for the continuing hemolysis.
    • These episodes are most frequently caused by parvovirus infection and usually resolve spontaneously within several days to a few weeks.
    • Parvovirus antibody studies are of limited value because a large proportion of the population has had parvovirus infection. However, molecular diagnostic tests are available to confirm the presence of parvovirus.
    • In addition, typical inclusions may be detected in early erythroid precursors in the bone marrow.
    • Sometimes, intravenous immunoglobulin treatment is necessary to attempt to resolve the parvovirus infection.
  • Hemoglobin C disease can coexist with sickle cell hemoglobin (hemoglobin SC disease), which can lead to hemolytic anemia and pain crises. Some patients may have vasoocclusive crises and aseptic necrosis of the femoral head.9,10

Prognosis

Patients have a normal life expectancy.

Patient Education

  • Patients may benefit from genetic counseling.
  • Inform patients that the disease has a benign course during pregnancy.

Miscellaneous

Medicolegal Pitfalls

  • Failure to offer genetic counseling
  • Failure to recommend ophthalmologic consult

Special Concerns

Pregnancy complications are rare.11

 


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
Further Reading
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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. Mantovani A, Figini I. Sickle cell-hemoglobin C retinopathy: transient obstruction of retinal and choroidal circulations and transient drying out of retinal neovessels. Int Ophthalmol. Apr 2008;28(2):135-7. [Medline].

  6. 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].

  7. Nagababu E, Fabry ME, Nagel RL, Rifkind JM. Heme degradation and oxidative stress in murine models for hemoglobinopathies: thalassemia, sickle cell disease and hemoglobin C disease. Blood Cells Mol Dis. Jul-Aug 2008;41(1):60-6. [Medline].

  8. Tokumasu F, Nardone GA, Ostera GR, et al. Altered membrane structure and surface potential in homozygous hemoglobin C erythrocytes. PLoS One. 2009;4(6):e5828. [Medline]. [Full Text].

  9. Tripette J, Alexy T, Hardy-Dessources MD, et al. Red blood cell aggregation, aggregate strength and oxygen transport potential of blood are abnormal in both homozygous sickle cell anemia and sickle-hemoglobin C disease. Haematologica. Aug 2009;94(8):1060-5. [Medline]. [Full Text].

  10. Gabrovsky A, Aderinto A, Spevak M, et al. Low dose, oral epsilon aminocaproic acid for renal papillary necrosis and massive hemorrhage in hemoglobin SC disease. Pediatr Blood Cancer. Jan 2010;54(1):148-50. [Medline].

  11. 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].

  12. 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].

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

  14. 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].

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

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

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Further Reading

Clinical guidelines
Screening for sickle cell disease in newborns: U.S. Preventive Services Task Force recommendation statement.
United States Preventive Services Task Force - Independent Expert Panel. 1996 (revised 2007). 10 pages. NGC:005908

Hemoglobinopathies in pregnancy.
American College of Obstetricians and Gynecologists - Medical Specialty Society. 2007 Jan. 9 pages. NGC:005700


Clinical trial

Natural History of Sickle Cell Disease and Other Hemolytic Disorders

Related eMedicine topics
Hematologic Disease and Pregnancy

Sickle Cell Anemia

Thalassemia

Retinopathy, Hemoglobinopathies

Cholecystitis

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Keywords

hemoglobin C disease, hemoglobin C, sickle cell anemia, hemolytic anemia, hemoglobin sickle, sickle cell anemia malaria, splenomegaly, cholelithiasis, gallstones, hemoglobinopathy, HbC disease, hemoglobinopathies, angioid streaks, hemoglobin C, Bruch membrane

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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: eMedicine Salary Employment

Managing Editor

Ronald A Sacher, MB, BCh, MD, FRCPC, Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health 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, Southwest Medical Consultants, SC, Department of Medicine, 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.

 
 
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