To the Editor: Although the appearance of the blood smear presented by Fairhurst and Casella (June 24 issue)1 is consistent with the presence of hemoglobin C disease, the extremely low mean corpuscular volume and the clinical scenario are more consistent with the presence of hemoglobin C β-thalassemia. Hemoglobin C β-thalassemia is common in African populations, and the clinical disorder is usually mild; features include low-grade anemia, a low mean corpuscular volume, and minimal elevation of the reticulocyte count, without splenomegaly. The blood smear is indistinguishable from that in hemoglobin C disease and shows microspherocytes and target cells.2 The electrophoretic pattern . . .
Abstract BY THE USE of filter-paper electrophoresis, the hematologist has gained a relatively simple and accurate method of differentiating the various abnormal or atypical hemoglobins.* Thus, we may identify normal hemoglobin (A), sickle hemoglobin (S), the combination of these (S-A) and C-hemoglobin in its homozygous (C-C) and heterozygous (C-A, C-S) states. From a practical standpoint, anemias which previously were undiagnosed or considered to be atypical sickle-cell anemia may be recognized now. It is the purpose of this paper to add to the literature another case of homozygous hemoglobin-C disease which was of particular interest because of the relatively long documented course of the illness, which prior to paper electrophoresis had been undiagnosed. The patient was followed intermittently from 1948 (when she first presented herself for treatment, with symptoms suggestive of gall-bladder disease) to 1953, when the syndrome was recognized and confirmed by paper electrophoresis. METHOD OF ELECTROPHORESIS A small tank was References 1. Spaet, T.: Identification of Abnormal Hemoglobins by Means of Paper Electrophoresis , J. Lab. & Clin. Med. 41:1, 1953. 2. Schneider, R. G.: Paper Electrophoresis of Hemoglobin as a Practical Method of Differentiating Various Types of Sickle Cell Disease and of Hemoglobin C Trait , Texas Rep. Biol & Med. 11:2, 1953. 3. Smith, E. W., and Conley, C. L.: Filter Paper Electrophoresis of Human Hemoglobins with Special Reference to the Incidence and Clinical Significance of Hemoglobin C , Bull. Johns Hopkins Hosp. 93:94, 1953. 4. Singer, K.; Chernoff, A. I., and Singer, L.: Studies on Abnormal Hemoglobins: 1. Their Demonstration in Sickle Cell Anemia and Other Hematologic Disorders by Means of Alkali Denaturation , Blood 5:413, 1951. 5. Larson, D. L., and Ranney, H. M.: Filter Paper Electrophoresis of Human Hemoglobin , J. Clin. Invest. 32:1070, 1953. 6. Spaet, T. H.; Alway, R. H., and Ward, G.: Homozygous Type C Hemoglobin , Pediatrics 12: 483, 1953. 7. Ranney, H. M.; Larson, D. L., and McCormack, G. H., Jr.: Some Clinical, Biochemical and Genetic Observations on Hemoglobin C , J. Clin. Invest. 32:1277, 1953. 8. Terry, D. W.; Motulsky, A. G., and Rath, C. E.: Homozygous Hemoglobin C , New England J. Med. 251:365, 1954.
A Ghanaian child on routine screening had a hemoglobin of 9.0 g per deciliter, a hematocrit of 24.3 percent, a mean corpuscular volume of 53.8 μm, and a reticulocyte count of 1.6 percent.