Images in Clinical Medicine
Homozygous Hemoglobin C Disease
N Engl J Med 2004; 350:e24June 24, 2004
- Article
A Ghanaian child was found on routine screening to have a hemoglobin level of 9.0 g per deciliter, a hematocrit of 24.3 percent, a mean corpuscular volume of 53.8 μm3, a red-cell distribution width of 28.8 percent, and an uncorrected reticulocyte count of 1.6 percent. There were no signs of splenomegaly. Testing of the father, who was asymptomatic, revealed only hemoglobin C on electrophoresis and a complete blood count similar to that of the child. A blood smear showed a constellation of findings that were characteristic of homozygous hemoglobin C disease: target cells (arrows), microspherocytes (arrowheads), rod-shaped cells containing hemoglobin C crystals (asterisk), anisocytosis, and poikilocytosis. The child is now healthy and asymptomatic.
Homozygous hemoglobin C disease is generally a benign condition associated with mild hemolytic anemia and splenomegaly. In terms of geographic distribution, the hemoglobin C allele is found at the highest frequencies in West Africa, where it has been associated with protection against malaria.
Rick M. Fairhurst, M.D., Ph.D.
National Institute of Allergy and Infectious Diseases, Bethesda, MD 20892James F. Casella, M.D.
Johns Hopkins University School of Medicine, Baltimore, MD 21205- Citing Articles (3)
Citing Articles
1
Katherine A. Poehling, Laney S. Light, Melissa Rhodes, Beverly M. Snively, Natasha B. Halasa, Ed Mitchel, William Schaffner, Allen S. Craig, Marie R. Griffin. (2010) Sickle Cell Trait, Hemoglobin C Trait, and Invasive Pneumococcal Disease. Epidemiology 21:3, 340-346
CrossRef2
Nathaniel J. Brittain, Cindy Erexson, Lawrence Faucette, Jerrold Ward, Hisashi Fujioka, Thomas E. Wellems, Rick M. Fairhurst. (2007) Non-opsonising aggregates of IgG and complement in haemoglobin C erythrocytes. British Journal of Haematology 136:3, 491-500
CrossRef3
(2004) Hemoglobin C Disease. New England Journal of Medicine 351:15, 1577-1577
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