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. 1970 Apr;49(4):666–672. doi: 10.1172/JCI106278

Red cell survival studies in hereditary spherocytosis

James S Wiley 1,2
PMCID: PMC322521  PMID: 5443169

Abstract

The in vivo survival of hereditary spherocytes has been investigated with the 51Cr red cell survival technique. Spherocytes invariably showed a shorter survival in a normal recipient than in the donor's own circulation. Moreover transfusion of spherocytes from a mother to her son, both with hereditary spherocytosis, showed that “foreign” spherocytes have a shorter survival than the “self” spherocytes of the recipient. These findings are attributed to splenic reticuloendothelial blockade, specific towards hereditary spherocytes in the patient's own circulation. There was no correlation between the Na+ influx and the hemolytic tendency of hereditary spherocytes when the latter was measured by the survival t½ in compatible normal recipients. However osmotic fragility influenced cell survival since cells with high fragility were rapidly sequestered in the normal spleen. It is suggested that both cell shape and a selective reticuloendothelial blockade in the spleen are important factors in determining the severity of hemolysis in the individual patient.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. BERTLES J. F. Sodium transport across the surface membrane of red blood cells in hereditary spherocytosis. J Clin Invest. 1957 Jun;36(6 Pt 1):816–824. doi: 10.1172/JCI103487. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Chapman R. G., McDonald L. L. Red cell life span after splenectomy in hereditary spherocytosis. J Clin Invest. 1968 Oct;47(10):2263–2267. doi: 10.1172/JCI105911. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Cooper R. A., Jandl J. H. The role of membrane lipids in the survival of red cells in hereditary spherocytosis. J Clin Invest. 1969 Apr;48(4):736–744. doi: 10.1172/JCI106031. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Drutz D. J., Koenig M. G., Rogers D. E. Further observations on the mechanism of reticuloendothelial blockade. J Exp Med. 1967 Dec 1;126(6):1087–1098. doi: 10.1084/jem.126.6.1087. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. GLYNN I. M. Sodium and potassium movements in human red cells. J Physiol. 1956 Nov 28;134(2):278–310. doi: 10.1113/jphysiol.1956.sp005643. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. JACOB H. S., JANDL J. H. INCREASED CELL MEMBRANE PERMEABILITY IN THE PATHOGENESIS OF HEREDITARY SPHEROCYTOSIS. J Clin Invest. 1964 Aug;43:1704–1720. doi: 10.1172/JCI105046. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. JANDL J. H., FILES N. M., BARNETT S. B., MACDONALD R. A. PROLIFERATIVE RESPONSE OF THE SPLEEN AND LIVER TO HEMOLYSIS. J Exp Med. 1965 Aug 1;122:299–326. doi: 10.1084/jem.122.2.299. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. JANDL J. H., GREENBERG M. S., YONEMOTO R. H., CASTLE W. B. Clinical determination of the sites of red cell sequestration in hemolytic anemias. J Clin Invest. 1956 Aug;35(8):842–867. doi: 10.1172/JCI103338. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. JANDL J. H., SIMMONS R. L., CASTLE W. B. Red cell filtration and the pathogenesis of certain hemolytic anemias. Blood. 1961 Aug;18:133–148. [PubMed] [Google Scholar]
  10. Jacob H. S. Membrane lipid depletion in hyperpermeable red blood cells: its role in the genesis of spherocytes in hereditary spherocytosis. J Clin Invest. 1967 Dec;46(12):2083–2094. doi: 10.1172/JCI105695. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Jandl J. H., Aster R. H. Increased splenic pooling and the pathogenesis of hypersplenism. Am J Med Sci. 1967 Apr;253(4):383–398. doi: 10.1097/00000441-196704000-00001. [DOI] [PubMed] [Google Scholar]
  12. Jensson O., Jónasson T., Olafsson O. Hereditary elliptocytosis in Iceland. Br J Haematol. 1967 Nov;13(6):844–854. doi: 10.1111/j.1365-2141.1967.tb08855.x. [DOI] [PubMed] [Google Scholar]
  13. KOENIG M. G., HEYSSEL R. M., MELLY M. A., ROGERS D. E. THE DYNAMICS OF RETICULOENDOTHELIAL BLOCKADE. J Exp Med. 1965 Jul 1;122:117–142. doi: 10.1084/jem.122.1.117. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Kinlough R. L., Bennett R. C., Lander H. The place of splenectomy in haematological disorders: the value of 51Cr techniques. Med J Aust. 1966 Nov 26;2(22):1022–1027. doi: 10.5694/j.1326-5377.1966.tb73787.x. [DOI] [PubMed] [Google Scholar]
  15. Krueger H. C., Burgert E. O., Jr Hereditary spherocytosis in 100 children. Mayo Clin Proc. 1966 Dec;41(12):821–830. [PubMed] [Google Scholar]
  16. MOLLISON P. L. The reticulo-endothelial system and red cell destruction. Proc R Soc Med. 1962 Nov;55:915–920. [PMC free article] [PubMed] [Google Scholar]
  17. MOLLISON P. L., VEALL N. The use of the isotope 51Cr as a label for red cells. Br J Haematol. 1955 Jan;1(1):62–74. doi: 10.1111/j.1365-2141.1955.tb05489.x. [DOI] [PubMed] [Google Scholar]
  18. Pearson H. A. The genetic basis of hereditary elliptocytosis with hemolysis. Blood. 1968 Dec;32(6):972–978. [PubMed] [Google Scholar]
  19. READ R. C., WILSON G. W., GARDNER F. H. The use of radioactive sodium chromate to evaluate the life span of the red blood cell in health and certain hematologic disorders. Am J Med Sci. 1954 Jul;228(1):40–52. doi: 10.1097/00000441-195407000-00005. [DOI] [PubMed] [Google Scholar]
  20. SCHLOESSER L. L., KORST D. R., CLATANOFF D. V., SCHILLING R. F. Radioactivity over the spleen and liver following the transfusion of chromium51-labelled erythrocytes in hemolytic anemia. J Clin Invest. 1957 Oct;36(10):1470–1485. doi: 10.1172/JCI103544. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Schapiro R. L., MacIntyre W. J., Schapiro D. I. The effect of homologous and heterologous carrier on the clearance of colloidal material by the reticuloendothelial system. J Lab Clin Med. 1966 Aug;68(2):286–299. [PubMed] [Google Scholar]
  22. Serjeant G. R., Richards R., Barbor P. R., Milner P. F. Relatively benign sickle-cell anaemia in 60 patients aged over 30 in the West Indies. Br Med J. 1968 Jul 13;3(5610):86–91. doi: 10.1136/bmj.3.5610.86. [DOI] [PMC free article] [PubMed] [Google Scholar]
  23. WAGNER H. N., Jr, IIO M. STUDIES OF THE RETICULOENDOTHELIAL SYSTEM (RES). 3. BLOCKADE OF THE RES IN MAN. J Clin Invest. 1964 Aug;43:1525–1532. doi: 10.1172/JCI105029. [DOI] [PMC free article] [PubMed] [Google Scholar]
  24. Weed R. I., LaCelle P. L., Merrill E. W. Metabolic dependence of red cell deformability. J Clin Invest. 1969 May;48(5):795–809. doi: 10.1172/JCI106038. [DOI] [PMC free article] [PubMed] [Google Scholar]

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