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Amelioration of Sickle Cell Disease by Persistent Fetal Hemoglobin

John F. Jackson, M.D.; Janie L. Odom; Warren N. Bell, M.D.
[+] Author Affiliations

Jackson, Miss.

From the Department of Clinical Laboratory Sciences, University of Mississippi Medical Center. Dr. Jackson is now at Tulane University of Louisiana, New Orleans.


JAMA. 1961;177(12):867-869. doi:10.1001/jama.1961.73040380028011b
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THE CLINICAL manifestations of sickle cell anemia are thought to be due to the abnormal hemoglobin in the erythrocytes. In addition to Hemoglobin S, however, the erythrocytes of patients with sickle cell anemia may also contain fetal hemoglobin.1 Since sickling of erythrocytes is decreased by fetal hemoglobin,2 this study was undertaken to evaluate the effect of persistence of significant amounts of fetal hemoglobin on the clinical severity of the manifestations of sickle cell anemia.

Materials and Methods  Hemoglobin electrophoresis was performed on the red cell hemolysates of all University of Mississippi Medical Center patients suspected of having sickle cell anemia or trait. The filter paper electrophoresis technique of Motulsky et al.3 was used. This method was modified by decreasing the voltage from 260 to 120 and increasing the duration of electrophoresis run from 5 to 23 hours, thereby achieving a greater spread of resulting components. Fetal

REFERENCES

Chernoff, A. I.:  Medical Progress; Human Hemoglobins in Health and Disease , New Engl J Med 253:365-374 ( (Sept. 1) ) 1955;.
Shields, G. S., et al.:  Studies in Sickle-Cell Disease. I. Quantitative Aspects of Sickling in Newborn Period , Pediatrics 22:309-318 ( (Aug.) ) 1958;.
Motulsky, A. G.; Paul, M. H.; and Durrum, E. L.:  Paper Electrophoresis of Abnormal Hemoglobins and Its Clinical Applications , Blood 9:897-910 ( (Sept.) ) 1954;.
Singer, K.; Chernoff, A. I.; and Singer, L.:  Studies on Abnormal Hemoglobins; I. Their Demonstration in Sickle Cell Anemia and Other Hematologic Disorders by Means of Alkali Denaturation , Blood 6:413-428 ( (May) ) 1951;.
Beaven, G. H.; Ellis, M. J.; and White, J. C.:  Studies on Human Foetal Haemoglobin. I. Detection and Estimation , Brit J Haemat 6:1-22 ( (Jan.) ) 1960;.
Itano, H. A.:  A Third Abnormal Hemoglobin Associated with Hereditary Hemolytic Anemia , Proc Nat Acad Sci 37:775-784 ( (Dec. 15) ) 1951;.
Singer, K.; Singer, L.; and Goldberg, S. R.:  Studies on Abnormal Hemoglobins. XI. Sickle-Cell-Thalassemia Disease in Negro , Blood 10:405-415 ( (May) ) 1955;.
Herman, E. C., and Conley, C. L.:  Hereditary Persistence of Fetal Hemoglobin; Family Study , Amer J Med 29:9-17 ( (July) ) 1960;.
Harris, J. W., et al.:  Studies on Destruction of Red Blood Cells. X. Biophysics and Biology of Sickle-Cell Disease , Arch Intern Med 97:145-168 ( (Feb.) ) 1956;.
Chernoff, A. I.:  Some Genetic Considerations of Abnormal Hemoglobins in Light of Their Amino Acid Structure , Amer J Hum Genet 13:151-170 ( (March) ) 1961;.
Sprague, C. C., and Paterson, J. C.:  Role of Spleen and Effect of Splenectomy in Sickle Cell Disease , Blood 13:569-581 ( (June) ) 1958;.
Smith, C. H. (New York), et al.:  Studies in Mediterranean (Cooley's) Anemia. I. Clinical and Hematologic Aspects of Splenectomy, with Special Reference to Fetal Hemoglobin Synthesis , Blood 10:582-599 ( (June) ) 1955;.
Rucknagel, D. L., and Chernoff, A. I.:  Immunologic Studies of Hemoglobins. III. Fetal Hemoglobin Changes in Circulation of Pregnant Women , Blood 10:1092-1099 ( (Nov.) ) 1955;.
Allen, D. W., and Jandl, J. H.:  Factors Influencing Relative Rates of Synthesis of Adult and Fetal Hemoglobin in Vitro , J Clin Invest 39:1107-1113 ( (July) ) 1960;.

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Chernoff, A. I.:  Medical Progress; Human Hemoglobins in Health and Disease , New Engl J Med 253:365-374 ( (Sept. 1) ) 1955;.
Shields, G. S., et al.:  Studies in Sickle-Cell Disease. I. Quantitative Aspects of Sickling in Newborn Period , Pediatrics 22:309-318 ( (Aug.) ) 1958;.
Motulsky, A. G.; Paul, M. H.; and Durrum, E. L.:  Paper Electrophoresis of Abnormal Hemoglobins and Its Clinical Applications , Blood 9:897-910 ( (Sept.) ) 1954;.
Singer, K.; Chernoff, A. I.; and Singer, L.:  Studies on Abnormal Hemoglobins; I. Their Demonstration in Sickle Cell Anemia and Other Hematologic Disorders by Means of Alkali Denaturation , Blood 6:413-428 ( (May) ) 1951;.
Beaven, G. H.; Ellis, M. J.; and White, J. C.:  Studies on Human Foetal Haemoglobin. I. Detection and Estimation , Brit J Haemat 6:1-22 ( (Jan.) ) 1960;.
Itano, H. A.:  A Third Abnormal Hemoglobin Associated with Hereditary Hemolytic Anemia , Proc Nat Acad Sci 37:775-784 ( (Dec. 15) ) 1951;.
Singer, K.; Singer, L.; and Goldberg, S. R.:  Studies on Abnormal Hemoglobins. XI. Sickle-Cell-Thalassemia Disease in Negro , Blood 10:405-415 ( (May) ) 1955;.
Herman, E. C., and Conley, C. L.:  Hereditary Persistence of Fetal Hemoglobin; Family Study , Amer J Med 29:9-17 ( (July) ) 1960;.
Harris, J. W., et al.:  Studies on Destruction of Red Blood Cells. X. Biophysics and Biology of Sickle-Cell Disease , Arch Intern Med 97:145-168 ( (Feb.) ) 1956;.
Chernoff, A. I.:  Some Genetic Considerations of Abnormal Hemoglobins in Light of Their Amino Acid Structure , Amer J Hum Genet 13:151-170 ( (March) ) 1961;.
Sprague, C. C., and Paterson, J. C.:  Role of Spleen and Effect of Splenectomy in Sickle Cell Disease , Blood 13:569-581 ( (June) ) 1958;.
Smith, C. H. (New York), et al.:  Studies in Mediterranean (Cooley's) Anemia. I. Clinical and Hematologic Aspects of Splenectomy, with Special Reference to Fetal Hemoglobin Synthesis , Blood 10:582-599 ( (June) ) 1955;.
Rucknagel, D. L., and Chernoff, A. I.:  Immunologic Studies of Hemoglobins. III. Fetal Hemoglobin Changes in Circulation of Pregnant Women , Blood 10:1092-1099 ( (Nov.) ) 1955;.
Allen, D. W., and Jandl, J. H.:  Factors Influencing Relative Rates of Synthesis of Adult and Fetal Hemoglobin in Vitro , J Clin Invest 39:1107-1113 ( (July) ) 1960;.
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