Thromb Haemost 1958; 02(03/04): 279-289
DOI: 10.1055/s-0038-1656279
Originalarbeiten — Original Articles — Travaux Originaux
Schattauer GmbH

A New Approach to the Thrombocytopathies. Thrombocytopathy A

Shirley A. Johnson
,
Raymond W. Monto
,
M. June Caldwell
This research was supported, in part, by a grant from the Michigan Heart Association.
Further Information

Publication History

Publication Date:
07 June 2018 (online)

Summary

A group of patients with clinical hemorrhagic disease who exhibited a prolonged bleeding time and a short prothrombin consumption time classified as Thrombocytopathy A were studied utilizing procedures involving isolated platelets and purified prothrombin. These frozen and thawed platelet extracts had poor platelet factor 3 activity which was normal after the extracts had been treated with ultrasonic oscillations. The electron microscope studies of the morphology of these platelets were abnormal. It is concluded that these platelets contain adequate amounts of platelet factor 3 but are resistant to disintegration and the activity is only liberated with difficulty.

The authors wish to thank Miss Jeanne M. Riddle, M.S. medical technologist in Hematology at Henry Ford Hospital for technical assistance in providing the platelet counts for this investigation.

 
  • References

  • 1 Singer K, and Ramot B. Pseudohemophilia Type B. Arch, intern. Med. 97: 715-725 1956;
  • 2 Braunsteiner J, and Pakesch F. Thrombocytoasthenia and Thrombocyto-pathia. Blood 11: 965-976 1956;
  • 3 Macfariane R. G. Critical review: The Mechanism of Haemostasis, Quart. J. Med. 10: 1-29 1941;
  • 4 Estren S, Medal L. S, and Dameshek W. Pseudohemophilia. Blood 1: 504-533 1946;
  • 5 Van Creveld S, and Paulssen M. M. P. A Form of Haemorrhagic Diathesis Characterized by the Lack of the Third Clotting Factor, Normally Present in Blood — Platelets (Thrombopathia haemophilica). Ann. paed. (Basel) 181: 193-199 1953;
  • 6 Buchanan J. D, and Leaveil B. S. Pseudohemophilia: Report of 13 New Cases and Statistical Review of Previously Reported Cases. Ann. intern. Med. 44: 241-256 1956;
  • 7 Johnson S. A, Caldwell M. J, and Monto R. W. The Blood Coagulation Mechanisms in Thrombocytopenic Blood. Thromb. Diath. haem. 1: 433-444 1957;
  • 8 Ware A. G, Fahey J. L, and Seegers W. H. Platelet Extracts, Fibrin Formation and Interaction of Purified Prothrombin and Thromboplastin. Amer. J. Physiol. 154: 140-147 1948;
  • 9 Van Creveld S, and Paulssen M. M. P. Isolation of and Properties of the Third Clotting Factor in Blood Platelets. Lancet 262: 23-25 1952;
  • 10 Jürgens R. Die Blutplättchen und ihre Bedeutung für Blutungsneigung und Thrombusbildung. Dtsch. med. Wschr. 77: 1265-1266 1952;
  • 11 Johnson S. A, Smathers W. M, and Schneider C. L. Platelets and Their Plasma Cofactor Activity in the Activation of Purified Prothrombin. Amer. J. Physiol. 170: 631-635 1952;
  • 12 Deutsch E, Johnson S. A, and Seegers W. J. Differentiation of Certain Platelet Factors Related to Blood Coagulation. Circulation Res. 3: 11-115 1955;
  • 13 Lee P. H, Johnson S. A, and Seegers W. J. Platelet Cothromboplastin: A Platelet Factor (s) Related to the Blood Clotting Mechanismus. Thromb. Diath. haem. 1: 16-26 1957;
  • 14 Soulier J. P, and Larrieu M. J. Syndrome de Willebrand — Jürgens et Throm-bopathies. Rev. Hémat. 9: 77-122 1954;
  • 15 Quick A. J. Nature of Bleeding in Jaundice. J. Amer. med. Ass. 110: 1658-1662 1938;
  • 16 Frommeyer W. B, and Epstein R. D. Medical Progress: Haemorrhagic Diseases. New. Engl. J. Med. 241: 700-712 1949;
  • 17 Hartman R. C, and Coniey C. L. Clot Retraction as a Measure of Platelet Function. Bull. Johns Hopk. Hosp. 93: 335-369 1953;
  • 18 Brecher G, Schneiderman M, and Cronkite E. P. The Reproducibility and Constancy of the Platelet Count. Am. J. clin. Path. 23: 15-26 1953;
  • 19 Sussman L. M, Cohen I. B, and Gittler R. Clinical Application of Simplified Serum Prothrombin Consumption Test. J. Amer. med. Ass. 156: 702-706 1954;
  • 20 Ware A. G, and Seegers W. H. Two-stage Procedure for the Quantitative Determination of Prothrombin Concentration. Am. J. clin. Path. 19: 471-482 1949;
  • 21 Biggs R, Douglas A. S, and Macfarlane R. G. Formation of Thromboplastin in Human Blood. J. Physiol. (Lond.) 119: 89-101 1953;
  • 22 Alkjaersig N, Abe T, and Seegers W. H. Purification and Quantitative Determination of Platelet Factor 3. Amer. J. Physiol. 181: 304-308 1955;
  • 23 Seegers W. H, and Smith J. P. Factors Which Influence the Activity of Purified Thrombin. Amer. J. Physiol. 137: 348-354 1942;
  • 24 Murray M, Johnson S. A, and Seegers W. H. Relationship of Certain Antihistamine Drugs to the Activation of Purified Prothrombin. Amer. J. Physiol. 178: 10-16 1954;
  • 25 Seegers W. H, Loomis E. C, and Vandenbelt J. M. Preparation of Prothrombin Products: Isolation of Prothrombin Properties. Arch. Biochem. 6: 85-95 1945;
  • 26 Seegers W. H. The Purification of Prothrombin. Record of Chemical Progress. 143—152 1953
  • 27 Schneider C. L, Claxton E. B, Hughes C. H, and Johnson S. A. Bovine Platelets in Large Quantities. Amer. J. Physiol. 179: 236-242 1954;
  • 28 Mertz E. T, and Owen C. A. Imidazole Buffer: Its Use in Blood Clotting Studies. Proc. Soc. exp. Biol. 43: 204-205 1940;
  • 29 Johnson S. A, Caldwell M. J, and Priest E. M. The Effect of the Oral Anticoagulant, “Marcumar” on Prothrombin and Related Components of the Blood Coagulation Mechanisms. Circulation Res. 5: 252-256 1957;
  • 30 Seegers W. H, Alkjaersig M, and Johnson S. A. Formation of Auto-prothrombin in Solutions Containing Purified Prothrombin and Purified Platelet Factor 3. Amer. J. Physiol. 181: 589-594 1955;
  • 31 Seegers W. H, and Johnson S. A. Conversion of Prothrombin to Autopro-thrombin II (Platelet Cofactor II) and Its Relation to the Blood Clotting Mechanisms. Amer. J. Physiol. 184: 259-264 1956;
  • 32 Alkjaersig N, Abe T, Johnson S. A, and Seegers W. H. An Accelerator of Prothrombin Activation Derived from Prothrombin. Amer. J. Physiol. 182: 443-446 1955;
  • 33 Link K. P. The Anticoagulant from Spoiled Sweet Clover Hay. Harvey Lect. 39: 162-216 1943;
  • 34 Mann F. D, and Hurn M. Complex Mechanisms of the Quick Prothrombin Test and the Effect of Dicumarol. Amer. J. clin. Path. 20: 225-232 1950;
  • 35 Johnson S. A, and Seegers W. H. The Reduction of Autoprothrombin II Activity with Dicumarol. Circulation Res. 4: 182-186 1956;
  • 36 Bonnin J. A. Platelet Functional Defects in Thrombocytopenic Purpura. Blood 12: 726-732 1957;