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XIII. Observations on the Role of pH in the Pathogenesis and Treatment of Painful Crisis in Sickle-Cell Disease

MORTIMER S. GREENBERG, M.D.; EDWARD H. KASS, M.D., Ph.D.

AMA Arch Intern Med. 1958;101(2):355-363.

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The chief clinical features of sickle-cell anemia are chronic hemolytic anemia and episodic painful crises; the underlying defect is the presence of S hemoglobin* in the red blood cells from patients with this disease. The deoxygenation of S hemoglobin leads to the formation of irregular elongated molecular masses—tactoids—which give to the red cells the distorted shape from which the disease derives its name.^1,5 The manner in which the sickling phenomenon accounts for the physiological and pathological disturbances that characterize sickle-cell anemia has been a major interest of Castle and his associates.^6,7

The chronic hemolytic anemia has been explained on the basis of the increased mechanical fragility that accompanies the sickled state,^6,8-10 with diminished life span of the erythrocytes in vivo.¹¹ The painful crises have been related to the increased viscosity of the blood that is proportional to the degree of sickling.^6,7 Factors which impede the . . . [Full Text PDF of this Article]

Author Affiliations
Boston

From the Thorndike Memorial Laboratory and Second and Fourth (Harvard) Medical Services, Boston City Hospital, and the Department of Medicine, Harvard Medical School.

Footnotes
Submitted for publication Sept. 30, 1957.

Present address of Dr. Greenberg: Lemuel Shattuck Hospital, Boston 30.

Presented in part to the 48th annual meeting of the American Society of Clinical Investigation, April 30, 1956.

This investigation was supported in part by grants from the National Institutes of Health, Public Health Service.

Many early observations suggested that it was the hemoglobin that was abnormal in sickle-cell anemia.^1-3 Pauling has on several public occasions acknowledged the stimulus to his studies of abnormal hemoglobins that came from Castle's suggestion to him that the hemoglobin probably was abnormal in sickle-cell anemia and that newer physical methods of study might detect the abnormality (see also Reference 4).

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