The property of the red blood cell to sickle is a manifestation of abnormal molecular arrangement of that cell's hemoglobin. This was established by Pauling and co-workers in 1949.1 Since then, other abnormal hemoglobins have been discovered by electrophoretic methods.2 The classification and nomenclature associated with sickle-cell disease has been standardized.3 Urologically, a discussion of hematological techniques and research is not important, but, clinically, association of hematuria with abnormal hemoglobin disease warrants more careful study and understanding of nomenclature than has been given in past urologic literature.
These facts have been established by electrophoretic methods:
1. Normal adult red blood cells contain normal hemoglobin designated as hemoglobin A.
2. Red blood cells which sickle may contain (a) an abnormal hemoglobin designated hemoglobin S, (b) a combination of abnormal hemoglobin S and normal hemoglobin A, (c) a combination of abnormal hemoglobin S and another abnormal hemoglobin designated as
GOLDMAN SM, CHAPMAN AZ, CROSS RR. The Hematuria of Abnormal Hemoglobin Diseases. AMA Arch Surg. 1955;71(6):881-884. doi:10.1001/archsurg.1955.01270180087011