Skip to main content
The American Journal of Pathology logoLink to The American Journal of Pathology
. 1972 Jul;68(1):57–66.

Effects of Normal and Aspirin Platelets on Defective Secondary Aggregation in the Hermansky-Pudlak Syndrome

A Test for Storage Pool Deficient Platelets

James G White, Carl J Witkop
PMCID: PMC2032614  PMID: 5080698

Abstract

Platelets from patients with the Hermansky-Pudlak syndrome (albinism, accumulation of ceroid-like pigment in bone marrow macrophages and mild hemorrhagic symptoms) and aspirin-treated normal platelets fail to develop irreversible secondary waves of aggregation when exposed to aggregating agents on the platelet aggregometer. The present study demonstrates that equal volumes of Hermansky-Pudlak and aspirin-treated platelets, when mixed together, respond in a biphasic manner comparable to normal cells. Correction of the release defect of aspirin platelets by storage pool deficient Hermansky-Pudlak cells indicates that different mechanisms are involved in the failure of the release reaction in the two cell types. The simple test may serve to distinguish other patients with storage pool deficient platelets from individuals who have taken aspirin or other drugs with an aspirin-like effect on platelet function.

Full text

PDF
57

Selected References

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

  1. BEDNAR B., HERMANSKY F., LOJDA Z. VASCULAR PSEUDOHEMOPHILIA ASSOCIATED WITH CEROID PIGMENTOPHAGIA IN ALBINOS. Am J Pathol. 1964 Aug;45:283–294. [PMC free article] [PubMed] [Google Scholar]
  2. Ball G., Fulwood M., Ireland D. M., Yates P. Effect of some inhibitors of platelet aggregation on platelet nucleotides. Biochem J. 1969 Sep;114(3):669–671. doi: 10.1042/bj1140669. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Clawson C. C., White J. G. Platelet interaction with bacteria. I. Reaction phases and effects of inhibitors. Am J Pathol. 1971 Nov;65(2):367–380. [PMC free article] [PubMed] [Google Scholar]
  4. Day H. J., Holmsen H. Concepts of the blood platelet release reaction. Ser Haematol. 1971;4(1):3–27. [PubMed] [Google Scholar]
  5. Doery J. C., Hirsh J., de Gruchy G. C. Aspirin: its effect on platelet glycolysis and release of adenosine diphosphate. Science. 1969 Jul 4;165(3888):65–67. doi: 10.1126/science.165.3888.65. [DOI] [PubMed] [Google Scholar]
  6. HERMANSKY F., PUDLAK P. Albinism associated with hemorrhagic diathesis and unusual pigmented reticular cells in the bone marrow: report of two cases with histochemical studies. Blood. 1959 Feb;14(2):162–169. [PubMed] [Google Scholar]
  7. Hardisty R. M., Hutton R. A. Bleeding tendency associated with "new" abnormality of platelet behaviour. Lancet. 1967 May 6;1(7497):983–985. doi: 10.1016/s0140-6736(67)92363-x. [DOI] [PubMed] [Google Scholar]
  8. Holmsen H., Day H. J., Stormorken H. The blood platelet release reaction. Scand J Haematol Suppl. 1969;8:3–26. [PubMed] [Google Scholar]
  9. Horowitz H. I., Stein I. M., Cohen B. D., White J. G. Further studies on the platelet-inhibitory effect of guanidinosuccinic acid and its role in uremic bleeding. Am J Med. 1970 Sep;49(3):336–345. doi: 10.1016/s0002-9343(70)80025-0. [DOI] [PubMed] [Google Scholar]
  10. Logan L. J., Rapaport S. I., Maher I. Albinism and abnormal platelet function. N Engl J Med. 1971 Jun 17;284(24):1340–1345. doi: 10.1056/NEJM197106172842402. [DOI] [PubMed] [Google Scholar]
  11. Muñiz F. J., Fradera J., Maldonado N., Perez-Santiago E. Albinism, bleeding tendency and abnormal pigmented cells in the bone marrow: a case report. Tex Rep Biol Med. 1970 Spring;28(1):167–173. [PubMed] [Google Scholar]
  12. Plague in Vietnam. Lancet. 1968 Apr 13;1(7546):799–800. [PubMed] [Google Scholar]
  13. Sahud M. A., Aggeler P. M. Platelet dysfunction--differentiation of a newly recognized primary type from that produced by aspirin. N Engl J Med. 1969 Feb 27;280(9):453–459. doi: 10.1056/NEJM196902272800901. [DOI] [PubMed] [Google Scholar]
  14. Weiss H. J., Aledort L. M. Impaired platelet-connective-tissue reaction in man after aspirin ingestion. Lancet. 1967 Sep 2;2(7514):495–497. doi: 10.1016/s0140-6736(67)91658-3. [DOI] [PubMed] [Google Scholar]
  15. Weiss H. J., Aledort L. M., Kochwa S. The effect of salicylates on the hemostatic properties of platelets in man. J Clin Invest. 1968 Sep;47(9):2169–2180. doi: 10.1172/JCI105903. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. Weiss H. J., Chervenick P. A., Zalusky R., Factor A. A familialdefect in platelet function associated with imapired release of adenosine diphosphate. N Engl J Med. 1969 Dec 4;281(23):1264–1270. doi: 10.1056/NEJM196912042812303. [DOI] [PubMed] [Google Scholar]
  17. White J. G. A biphasic response of platelets to serotonin. Scand J Haematol. 1970;7(3):145–151. doi: 10.1111/j.1600-0609.1970.tb01881.x. [DOI] [PubMed] [Google Scholar]
  18. White J. G., Edson J. R., Desnick S. J., Witkop C. J., Jr Studies of platelets in a variant of the Hermansky-Pudlak syndrome. Am J Pathol. 1971 May;63(2):319–332. [PMC free article] [PubMed] [Google Scholar]
  19. White J. G. Origin and function of platelet dense bodies. Ser Haematol. 1970;3(4):17–46. [PubMed] [Google Scholar]

Articles from The American Journal of Pathology are provided here courtesy of American Society for Investigative Pathology

RESOURCES