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Comment on Diagnosis and a Report on Splenectomy in Two Sisters

G. HILKOVITZ, M.B., B.Ch.; W. W. MARTIN, JR., M.D.

Arch Intern Med. 1961;108(1):109-113.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

Sickle-cell anemia is a chronic hemolytic anemia affecting persons of Negro descent who have inherited a gene from both parents¹ that controls the formation of an abnormal hemoglobin (hemoglobin S). This differs from normal hemoglobin (hemoglobin A) in amino-acid composition,² electrophoretic mobility,³ solubility,⁴ and affinity for oxygen.⁵ When deprived of oxygen, hemoglobin S undergoes molecular rearrangement,³ and the erythrocytes become altered in shape.

The diagnosis of sickle-cell anemia is established by:

1. hemoglobin electrophoresis, to demonstrate that hemoglobin S constitutes 80% or more of the total hemoglobin;
   
2. the alkali- denaturation test,⁶ to show that fetal hemoglobin (hemoglobin F) is present in a concentration not exceeding 20% of the total hemoglobin;
   
3. starch-gel electrophoresis,⁷ to demonstrate that hemoglobin A₂ is present in normal concentrations (less than 5.1%,8 and
   
4. the ferrohemoglobin solubility test,⁹ to distinguish between hemoglobins S and D which have the same electrophoretic mobility.
   

Author Affiliations
RICHMOND, VA.

From the Laboratory for Clinical Investigation, Medical College of Virginia.

Footnotes
Submitted for publication April 28, 1960.

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