In 1927, in a classic paper, Hahn and Gillespie 19 clearly defined the basis of the anomalous physical behavior of the sickle cell as an abnormality of its hemoglobin when in the deoxygenated state and pointed to the affected cells as the primary cause of the characteristic hemolytic anemia. From microscopic observations of drops of blood suspended in a gas chamber and from the results of the removal of a large spleen from a child with sicklemia, they were led to make the following statement: Sickle cell formation is a reversible phenomenon depending on the free or combined state of the hemoglobin of the susceptible corpuscles. When the hemoglobin is in the combined state, the discoid form is stable; when in the uncombined state, the distorted form is stable. The behavior of the susceptible corpuscles toward oxygen asphyxia thus constitutes a special application of a hypothesis correlating contour with
HARRIS JW, BREWSTER HH, HAM TH, CASTLE WB. Studies on the Destruction of Red Blood Cells: X. The Biophysics and Biology of Sickle-Cell Disease. AMA Arch Intern Med. 1956;97(2):145–168. doi:10.1001/archinte.1956.00250200021002
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: