A New Abnormality of Platelet Function

Summary

This report concerns seven patients with thrombopathy, one of whom, a patient with a moderately severe bleeding tendency, exhibited a type of platelet deficiency not previously recorded. There was indirect evidence of platelet insufficiency in the prothrombin consumption test, which was not substantiated by direct examination of platelet function in the thromboplastin generation test. Since no antiplatelet activity could be demonstrated in the plasma, we are proposing the existence of a plasmatic factor which is necessary for the normal function of platelets, a factor lacking in this patient’s plasma.

• References

• 1 Donné A. De l’origine des globules du sang, de leur mode de formation et de leur fin. Compt. rend. Acad. Sci. U: 366 1842;
• 2 Hayem G. Du caillot non rétractile: Suppression de la formation du sérum sanguin dans quelques états pathologiques. Compt. rend. Acad. Sci. 123: 894 1896;
• 3 Glanzmann E. Hereditäre hämorrhagische Thrombasthenie: Ein Beitrag zur Pathologie der Blutplättchen. Jb. Kinderheilk. 88: 113 1918;
• 4 v. Willebrand E. A. Hereditär pseudohemofili. Finska Läk.-Sällsk. Handl. 68: 87 1926;
• 5 v. Willebrand E. A. Über hereditäre Pseudohämophilie. Acta med. scand. 76: 521 1931;
• 6 v. Willebrand E. A, Jürgens R. Über ein neues vererbbares Blutungsübel: Die konstitutionelle Thrombopathie. Dtsch. Arch. klin. Med. 175: 453 1933;
• 7 MacFarlane R. G. Critical review: Mechanism of hemostasis. Quart. J. Med. 10: 1 1941;
• 8 Owen Jr CA, Mann F. D, Hurn MargaretM, Stickney J. M. Evaluation of disorders of blood coagulation in the clinical laboratory. Amer. J. clin. Path. 25: 1417 1955;
• 9 Quick A. J. On the quantitative estimation of prothrombin. Amer. J. clin. Path. 15: 560 1945;
• 10 Hum Margaret, Barker N. W, Magath T. B. The determination of the prothrombin time following the administration of Dicumarol, 3,3’-methylenebis (4-hydroxycoumarin), with special reference to thromboplastin. J. Lab. clin. Med. 30: 432 1945;
• 11 Biggs Rosemary, Douglas A. S. The thromboplastin generation test. J. clin. Path. 06: 23 1953;
• 12 Duckert F, Flückiger P, Isenschmid H, Matter M, Vogel-Meng J, Koller F. A modification of the thromboplastin generation test : Role of calcium, Factor V and Factor VII in prothrombin conversion initiated by active blood thromboplastin. Acta haemat. 12: 196 1954;
• 13 Owen Jr CA, Thompson Jr JH. Soybean phosphatides in prothrombin-consumption and thromboplastin-generation tests: Their use in recognizing “thrombasthenie hemophilia”. Amer. J. clin. Path. 33: 197 1960;
• 14 Quick A. J, Favre-Gilly J. E. The prothrombin consumption test: Its clinical and theoretic implications. Blood 04: 1281 1949;
• 15 Bergsagel E. E, Hougie C. Intermediate stages in the formation of blood thromboplastin. Brit. J. Haemat. 02: 113 1956;
• 16 Hougie Cecil. The role of factor V in the formation of blood thromboplastin. J. Lab. clin. Med. 50: 61 1957;
• 17 Fisch U, Duckert F. Some aspects of kinetics of the first stages of blood thromboplastin formation. Thrombos. Diathes. haemorrh. (Stuttg.) 03: 98 1959;
• 18 Johnson Shirley A, Monto R. W, Caldwell MJune. A new approach to the thrombocytopathies: Thrombocytopathy A. Thrombos. Diathes. haemorrh. (Stuttg.) 02: 279 1958;
• 19 Ulutin O. N. The Qualitative Platelet Diseases. In International Symposium on Blood Platelets. Boston, Little: Brown & Company; 1961: 553.
• 20 Cahalane S. F, Johnson ShirleyA, Monto R. W, Caldwell MJune. Acquired thrombocytopathy: Observations on the coagulation defect in uremia. Amer. J. clin. Path. 30: 507 1958;
• 21 Pachter M. R, Johnson ShirleyA, Neblett T. R, Truant J. P. Bleeding, platelets and macroglobulinemia. Amer. J. clin. Path. 31: 467 1959;
• 22 Pachter M. R, Johnson S. A, Basinski D. H. The effect of macroglobulins and their dissociation units on release of platelet factor 3. Thrombos. Diathes. haemorrh. (Stuttg.) 03: 501 1959;
• 23 Bowie EJW, Thompson Jr JH, Pascuzzi C. A, Owen Jr CA. Thrombosis in systemic lupus erythematosus despite circulating anticoagulants. J. Lab. clin. Med. 62: 416 1963;
• 24 Brinkhous K. M. Clotting defect in hemophilia: Deficiency in a plasma factor required for platelet utilization. Proc. Soc. exper. Biol. 66: 117 1947;