Skip to main content
NCBI home page
The Journal of Clinical Investigation logoLink to The Journal of Clinical Investigation
. 1981 Sep;68(3):631–638. doi: 10.1172/JCI110297

Exercise-induced hemolysis in xerocytosis. Erythrocyte dehydration and shear sensitivity.

O S Platt, S E Lux, D G Nathan
PMCID: PMC370843  PMID: 7276163

Abstract

A patient with xerocytosis was found to have swimming-induced intravascular hemolysis and shortening of erythrocyte life-span. In a microviscometer, xerocytes were more susceptible than normal erythrocytes to hemolysis by shear stress. Fractionation of normal and abnormal cells on discontinuous Stractan density gradients revealed that increasingly dehydrated cells were increasingly more shear sensitive. This sensitivity was partially corrected by rehydrating xerocytic erythrocytes by means of the cation-ionophore nystatin in a high potassium buffer. Conversely, normal erythrocytes were rendered shear sensitive by dehydrating them with nystatin in a low potassium buffer. This effect of dehydration was entirely reversible if normal cells were dehydrated for less than 4 h but was only partially reversed after more prolonged dehydration. It is likely that dehydration of erythrocytes results in shear sensitivity primarily because of concentration of cell contents and reduced cellular deformability. With prolonged dehydration, secondary membrane changes may potentiate the primary effect. This increased shear sensitivity of dehydrated cells may explain atraumatic exercise-induced hemolysis in xerocytosis as cardiac output is shifted to vessels of exercising muscles with small diameters and high shear rates.

Full text

PDF
631

Selected References

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

  1. Albala M. M., Fortier N. L., Glader B. E. Physiologic features of hemolysis axxociated with altered cation and 2,3-diphosphoglycerate content. Blood. 1978 Jul;52(1):135–141. [PubMed] [Google Scholar]
  2. BUCKLE R. M. EXERTIONAL (MARCH) HAEMOGLOBINURIA: REDUCTION OF HAEMOLYTIC EPISODES BY USE OF SORBO-RUBBER INSOLES IN SHOES. Lancet. 1965 May 29;1(7396):1136–1138. doi: 10.1016/s0140-6736(65)91958-6. [DOI] [PubMed] [Google Scholar]
  3. Banga J. P., Pinder J. C., Gratzer W. B., Linch D. C., Huehns E. R. An erythrocyte membrane-protein anomaly in march haemoglobinuria. Lancet. 1979 Nov 17;2(8151):1048–1049. doi: 10.1016/s0140-6736(79)92444-9. [DOI] [PubMed] [Google Scholar]
  4. Bienzle U., Bhadki S., Knüfermann H., Niethammer D., Kleihauer E. Abnormality of erythrocyte membrane protein in a case of congenital stomatocytosis. Klin Wochenschr. 1977 Jun 15;55(12):569–572. doi: 10.1007/BF01490509. [DOI] [PubMed] [Google Scholar]
  5. CROSBY W. H., FURTH F. W. A modification of the benzidine method for measurement of hemoglobin in plasma and urine. Blood. 1956 Apr;11(4):380–383. [PubMed] [Google Scholar]
  6. Cass A., Dalmark M. Equilibrium dialysis of ions in nystatin-treated red cells. Nat New Biol. 1973 Jul 11;244(132):47–49. doi: 10.1038/newbio244047a0. [DOI] [PubMed] [Google Scholar]
  7. Clark M. R., Mohandas N., Caggiano V., Shohet S. B. Effects of abnormal cation transport on deformability of desiccytes. J Supramol Struct. 1978;8(4):521–532. doi: 10.1002/jss.400080414. [DOI] [PubMed] [Google Scholar]
  8. Corash L. M., Piomelli S., Chen H. C., Seaman C., Gross E. Separation of erythrocytes according to age on a simplified density gradient. J Lab Clin Med. 1974 Jul;84(1):147–151. [PubMed] [Google Scholar]
  9. DAVIDSON R. J. EXERTIONAL HAEMOGLOBINURIA: A REPORT ON THREE CASES WITH STUDIES ON THE HAEMOLYTIC MECHANISM. J Clin Pathol. 1964 Sep;17:536–540. doi: 10.1136/jcp.17.5.536. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Davidson R. J. March or exertional haemoglobinuria. Semin Hematol. 1969 Apr;6(2):150–161. [PubMed] [Google Scholar]
  11. GILLIGAN D. R., ALTSCHULE M. D. March hemoglobinuria in a woman. N Engl J Med. 1950 Dec 14;243(24):944–948. doi: 10.1056/NEJM195012142432402. [DOI] [PubMed] [Google Scholar]
  12. Gilligan D. R., Altschule M. D., Katersky E. M. PHYSIOLOGICAL INTRAVASCULAR HEMOLYSIS OF EXERCISE. HEMOGLOBINEMIA AND HEMOGLOBINURIA FOLLOWING CROSS-COUNTRY RUNS. J Clin Invest. 1943 Nov;22(6):859–869. doi: 10.1172/JCI101460. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Glader B. E., Fortier N., Albala M. M., Nathan D. G. Congenital hemolytic anemia associated with dehydrated erythrocytes and increased potassium loss. N Engl J Med. 1974 Sep 5;291(10):491–496. doi: 10.1056/NEJM197409052911003. [DOI] [PubMed] [Google Scholar]
  14. Horvath S. M. Review of energetics and blood flow in exercise. Diabetes. 1979 Jan;28 (Suppl 1):33–38. doi: 10.2337/diab.28.1.s33. [DOI] [PubMed] [Google Scholar]
  15. Jacob H. S., Jandl J. H. A simple visual screening test for glucose-6-phosphate dehydrogenase deficiency employing ascorbate and cyanide. N Engl J Med. 1966 May 26;274(21):1162–1167. doi: 10.1056/NEJM196605262742102. [DOI] [PubMed] [Google Scholar]
  16. Jaffé E. R., Gottfried E. L. Hereditary nonspherocytic hemolytic disease associated with an altered phospholipid composition of the erythrocytes. J Clin Invest. 1968 Jun;47(6):1375–1388. doi: 10.1172/JCI105829. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Kaden W. S. Traumatic haemoglobinuria in conga-drum players. Lancet. 1970 Jun 20;760(1):1341–1342. doi: 10.1016/s0140-6736(70)91935-5. [DOI] [PubMed] [Google Scholar]
  18. LOCK S. P., SMITH R. S., HARDISTY R. M. Stomatocytosis: a hereditary red cell anomally associated with haemolytic anaemia. Br J Haematol. 1961 Jul;7:303–314. doi: 10.1111/j.1365-2141.1961.tb00341.x. [DOI] [PubMed] [Google Scholar]
  19. La Celle P. L., Weed R. I. The contribution of normal and pathologic erythrocytes to blood rheology. Prog Hematol. 1971;7(0):1–31. [PubMed] [Google Scholar]
  20. Mentzer W. C., Jr, Smith W. B., Goldstone J., Shohet S. B. Hereditary stomatocytosis: membrane and metabolism studies. Blood. 1975 Nov;46(5):659–669. [PubMed] [Google Scholar]
  21. Mentzer W. C., Lubin B. H., Emmons S. Correction of the permeability defect in hereditary stomatocytosis by dimethyl adipimidate. N Engl J Med. 1976 May 27;294(22):1200–1204. doi: 10.1056/NEJM197605272942202. [DOI] [PubMed] [Google Scholar]
  22. Miller D. R., Rickles F. R., Lichtman M. A., La Celle P. L., Bates J., Weed R. I. A new variant of hereditary hemolytic anemia with stomatocytosis and erythrocyte cation abnormality. Blood. 1971 Aug;38(2):184–204. [PubMed] [Google Scholar]
  23. Nevaril C. G., Lynch E. C., Alfrey C. P., Jr, Hellums J. D. Erythrocyte damage and destruction induced by shearing stress. J Lab Clin Med. 1968 May;71(5):784–790. [PubMed] [Google Scholar]
  24. Oski F. A., Naiman J. L., Blum S. F., Zarkowsky H. S., Whaun J., Shohet S. B., Green A., Nathan D. G. Congenital hemolytic anemia with high-sodium, low-potassium red cells. Studies of three generations of a family with a new variant. N Engl J Med. 1969 Apr 24;280(17):909–916. doi: 10.1056/NEJM196904242801701. [DOI] [PubMed] [Google Scholar]
  25. Snyder L. M., Lutz H. U., Sauberman N., Jacobs J., Fortier N. L. Fragmentation and myelin formation in hereditary xerocytosis and other hemolytic anemias. Blood. 1978 Oct;52(4):750–761. [PubMed] [Google Scholar]
  26. Streeton J. A. Traumatic haemogloinuria caused by karate exercises. Lancet. 1967 Jul 22;2(7508):191–192. doi: 10.1016/s0140-6736(67)90010-4. [DOI] [PubMed] [Google Scholar]
  27. Wiley J. S., Ellory J. C., Shuman M. A., Shaller C. C., Cooper R. A. Characteristics of the membrane defect in the hereditary stomatocytosis syndrome. Blood. 1975 Sep;46(3):337–356. [PubMed] [Google Scholar]
  28. Zarkowsky H. S., Oski F. A., Sha'afi R., Shohet S. B., Nathan D. G. Congenital hemolytic anemia with high sodium, low potassium red cells. I. Studies of membrane permeability. N Engl J Med. 1968 Mar 14;278(11):573–581. doi: 10.1056/NEJM196803142781101. [DOI] [PubMed] [Google Scholar]

Articles from Journal of Clinical Investigation are provided here courtesy of American Society for Clinical Investigation

RESOURCES