Platelet Coagulant Activities and Clinical Severity in Haemophilia

Summary

Assays for platelet coagulant activities concerned with the initiation and progress of intrinsic coagulation were done pair-wise in two groups of 16 haemophiliacs without detectable factor VIII. The patients in the ‘severe’ group had experienced 7.9-22.8 (mean 13.7) spontaneous haemorrhages per 100 days during an average of 431 days of observation, whereas those in the ‘mild’ group had bled 0-5.5 (mean 3.2) times per 100 days during an average of 282 days of observation. Contact product forming activity, collagen-induced coagulant activity and intrinsic factor-Xa forming activity were decreased in the severely-affected group and increased in the mildly affected group, whereas there was no great difference in platelet factor 3 activity between the two groups. Variations of platelet coagulant activities concerned with the initiation and early stages of intrinsic coagulation may influence clinical severity in haemophilia.

^* Present address, Department of Medicine, Temple University, School of Medicine, Health Sciences Center, 3400 North Broad Street, Philadelphia, Pennsylvania 19140 U.S.A.

• References

• 1 Biggs R, Denson K. W. E, Akman N, Borrett R, Haddon M. 1970; Antithrombin III, antifactor Xa and heparin. British Journal of Haematology 19: 283.
• 2 Biggs R, Macfarlane R. G. 1962. Human Blood Coagulation and its Disorders. Blackwell Scientific Publications; Oxford.:
• 3 Borchgrevink C. F. 1960; A method for measuring platelet adhesiveness in vivo. Acta Medica Scandinavica 168: 157.
• 4 Brecher G, Cronkite E. P. 1950; Morphology and enumeration of human blood platelets. Journal of Applied Physiology 03: 365.
• 5 Brustkhotjs K. M. 1947; Clotting defect in hemophilia: Deficiency in a plasma factor required for platelet utilization. Proceedings of the Society for Experimental Biology and Medicine 66: 117.
• 6 Call K. E, Mull M. M, Hathaway W. E. 1966; Platelet function in classic (AHF-deficiency) hemophilia : Report of a case with defective platelet function. Blood 33: 26.
• 7 Djerassi I, de yBiES A, Fisher I, Remez D. 1957; Concurrent hemophilia and qualitative platelet disturbance. Acta Haematologica 18: 354.
• 8 Eliakim M. 1962; Antihaemophilic globulin and plasma thromboplastin component deficiencies associated with a qualitative platelet disturbance. Acta Haematologica 28: 61.
• 9 Feissly R, Fried A. 1924; Die Blutplättchen des hämophilen Blutes. Klinische Wochenschrift 03: 831.
• 10 Fonio A. 1932; Die Unterkühlungs-Zentrifugier methode. Ein neues Verfahren zur Gerinnung von plättchenhaltigem und plättchenfreiem Plasma ohne gerinnungshemmende Zusätze. Als Beitrag zur Unt er suchungs - Me tho dik der Blutgerinnung. Zeitschrift für klinische Medizin 119: 687.
• 11 Holburn R. R, Carroll L. T, Tocantins L. M. 1957; Defective platelets as a cause of deficient generation of thromboplastin in a family of hemophilics. Federation Proceedings 16: 60.
• 12 Howell W. H, Chekada E. B. 1926; The cause of the delayed clotting of hemophilic blood. American Journal of Physiology 78: 500.
• 13 Ivy A. C, Nelson D, Bucher G. 1940; The standardization of certain factors in the cutaneous “venostasis” bleeding time technique. Journal of Laboratory and Clinical Medicine 26: 1812.
• 14 Minot G. R, Lee R. I. 1916; Blood platelets in hemophilia. Archives of Internal Medicine 17: 474.
• 15 Owen C. A, Bowie E. J. W, Thompson J. H, Didisheim P. 1971. Thrombocytopathy. In: Brinkhous K. M, Shermer R. W, Mostofi F. K. (eds.), The Platelet. Williams and Wilkins; Baltimore: 233.
• 16 Patek A. J, Stetson R. P. 1936; Hemophilia. I. The abnormal coagulation of the blood and its relation to the blood platelets. J ournal of Clinical Investigation 15: 531.
• 17 Quick A. J. 1947; Studies on the enigma of the hemostatic dysfunction of hemophilia. American Journal of the Medical Sciences 214: 272.
• 18 Salzman E. W. 1963; Measurement of platelet adhesiveness. A simple in vitro technique demonstrating an abnormality in von Willebrand’s disease. Journal of Laboratory and Clinical Medicine 62: 724.
• 19 Walsh P. N. (1972a): The effect of dilution of plasma on coagulation. The significance of the dilution -activation phenomenon for the study of platelet coagulant activities. British J ournal of Haematology 22: 219.
• 20 Walsh P. N. (1972b): The role of platelets in the contact phase of blood coagulation. British Journal of Haematology 22: 237.
• 21 Walsh P. N. 1972c The effects of collagen and kaolin on the intrinsic coagulant activity of platelets. Evidence for an alternative pathway in intrinsic coagulation not requiring factor XII. British Journal of Haematology 22: 393.
• 22 Walsh P. N. 1972d Platelet coagulant activities in thrombasthenia. British Journal of Haematology 23: 553.
• 23 Walsh P. N, Biggs R. 1972; The role of platelets in intrinsic factor-Xa formation. British J ournal of Haematology 22: 743.
• 24 Wright H. P. 1946; Adhesiveness of blood platelets in hemophilia. Lancet 01: 306.