Hemoglobin C Disease Follow-up

  • Author: Suzanne M Carter, MS; Chief Editor: Emmanuel C Besa, MD   more...
 
Updated: Jan 10, 2012
 

Further Inpatient Care

Admit patients if they require a cholecystectomy or splenectomy.

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Further Outpatient Care

Monitor patients for anemia.

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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.

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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.[11, 12]

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Prognosis

Patients have a normal life expectancy.

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Patient Education

Patients may benefit from genetic counseling.

Inform patients that the disease has a benign course during pregnancy.

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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.

Specialty Editor Board

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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 Association for the Advancement of Science, American Association of Blood Banks, American Clinical and Climatological Association, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists, International Society of Blood Transfusion, International Society on Thrombosis and Haemostasis, and Royal College of Physicians and Surgeons of Canada

Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

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, Jefferson Medical College of 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.

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. Alkindi S, Al Zadjali S, Al Madhani A, Daar S, Al Haddabi H, Al Abri Q, et al. Forecasting hemoglobinopathy burden through neonatal screening in Omani neonates. Hemoglobin. Jan 2010;34(2):135-44. [Medline].

  4. 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].

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

  6. 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].

  7. Leveziel N, Bastuji-Garin S, Lalloum F, Querques G, Benlian P, Binaghi M, et al. Clinical and laboratory factors associated with the severity of proliferative sickle cell retinopathy in patients with sickle cell hemoglobin C (SC) and homozygous sickle cell (SS) disease. Medicine (Baltimore). Nov 2011;90(6):372-8. [Medline].

  8. 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].

  9. 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].

  10. 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].

  11. 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].

  12. 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].

  13. 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].

  14. 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].

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

  16. 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].

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

  18. 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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