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Differential Diagnosis and Treatment in Hemorrhagic Disease

Differential Diagnosis and Treatment in Hemorrhagic Disease Abstract Differential diagnosis and treatment of excessive bleeding rest upon an understanding of normal hemostatic mechanisms. Although all of the intricacies of this complicated physiological process are not fully understood, enough information is available to form a theory. The simple diagram (Fig. 1) presents the main facets of normal hemostasis. The steps probably occur simultaneously but it is convenient to consider them in four sections: vascular, tissue, platelet and plasma. Steps in Normal Hemostasis Vascular. —Intact blood vessels are obviously necessary to contain the circulating blood. In addition, blood vessels, particularly those with muscular walls, respond to mechanical (injury) or chemical stimuli (see serotonin, below) with constriction, thus reducing blood flow to an injured area. Tissue. —Normal tissues play a multiple role. First, all of the many circulating factors important in maintenance of fluidity as well as formation of the blood clot are formed in various tissue sites (Table 1). Second, References 1. Dr. Oscar Ratnoff made it possible for us to see this patient. 2. Lewis, J. H.; Tagnon, H. J.; Davidson, C. S.; Minot, G. R., and Taylor, F. H. L.: The Relation of Certain Fractions of the Plasma Globulins to the Coagulation Defect in Hemophilia , Blood 1:166-172 ( (March) ) 1946. 3. Brinkhous, K. M.: Clotting Defect in Hemophilia: Deficiency in a Plasma Factor Required for Platelet Utilization , Proc. Soc. Exper. Biol. & Med. 66:117-120 ( (Oct.) ) 1947. 4. Quick, A. J.: Studies on the Enigma of the Hemostatic Dysfunction of Hemophilia , Am. J. M. Sc. 214:272-280 ( (Sept.) ) 1947. 5. Aggeler, P. M.; White, S. G.; Glendening, M. B.; Page, E. W.; Leake, T. B., and Bates, G.: Plasma Thromboplastin Component (PTC) Deficiency: A New Disease Resembling Hemophilia , Proc. Soc. Exper. Biol. & Med. 79:692-694 ( (April) ) 1952. 6. Lewis, J. H., and Ferguson, J. H.: Hemorrhagic Diathesis Due to PTC (Plasma Thromboplastin Component) Deficiency , Proc. Soc. Exper. Biol. & Med. 82:445-448 ( (March) ) 1953. 7. Biggs, R. G.; Douglas, A. S.; Macfarlane, R. G.; Dacie, J. V.; Pitney, W. R.; Merskey, C., and O'Brien, J. R.: Christmas Disease: A Condition Previously Mistaken for Haemophilia , Brit. M. J. 2:1378-1382 ( (Dec. 27) ) 1952. 8. Rosenthal, R. L.; Dreskin, O. H., and Rosenthal, N.: New Hemophilia-like Disease Caused by Deficiency of a Third Plasma Thromboplastin Factor , Proc. Soc. Exper. Biol. & Med. 82:171-174 ( (Jan.) ) 1953. 9. Spaet, T. H.; Aggeler, P. M., and Kinsell, B. G.: A Possible Fourth Plasma Thromboplastin Component , J. Clin. Invest. 33:1095-1102 ( (Aug.) ) 1954. 10. Ratnoff, O. D., and Colopy, J. E.: A Familial Hemorrhagic Trait Associated with a Deficiency of a Clot-Promoting Fraction of Plasma , J. Clin. Invest. 34:602-613 ( (April) ) 1955. 11. Seegers, W. H. and Alkjaersig, N.: The Preparation of Prothrombin Derivatives and an Indication of Their Properties . Arch. Biochem. and Biophysics 61:1-19 ( (March) ) 1956. 12. Owren, P. A.: Prothrombin and Accessory Factors: Clinical Significance Am. J. Med. 14: 201-215 ( (Feb.) ) 1953. 13. Alexander, B.; Goldstein, R.; Landwehr, G., and Cook, C. D.: Congenital SPCA Deficiency: A Hitherto Unrecognized Coagulation Defect with Hemorrhage Rectified by Serum and Serum Fractions , J. Clin. Invest. 30:596-608 ( (June) ) 1951. 14. Owren, P. A.: Parahæmophilia: Hemorrhagic Diathesis Due to Absence of a Previously Unknown Clotting Factor , Lancet 1:446-448 ( (April 5) ) 1947. 15. Ware, A. G., and Seegers, W. H.: Plasma Accelerator Globulin: Partial Purification, Quantitative Determination, and Properties , J. Biol. Chem. 172:699-705 ( (Feb.) ) 1948. 16. Quick, A. J., and Stefanini, M.: The Concentration of the Labile Factor of the Prothrombin Complex in Human, Dog, and Rabbit Blood: Its Significance in the Determination of Prothrombin Activity , J. Lab. & Clin. Med. 33:819-826 ( (July) ) 1948. 17. Koller, F.: Symposium: What is Hemophilia? Is Hemophilia a Nosologic Entity? Blood 9:286-290 ( (March) ) 1954. 18. Lewis, J. H.; Fresh, J. W., and Ferguson, J. H.: Congenital Hypoproconvertinemia , Proc. Soc. Exper. Biol. & Med. 84:651-654 ( (Dec.) ) 1953. 19. Hougie, C., and Graham, J. B.: The Blood Clotting Role and Mode of Inheritance of the Stuart Factor: A New Clotting Factor Distinct from SPCA, Proceedings of the 6th Congress of the International Society for Blood Transfusion, Boston, September, 1956, pp. 80-81. 20. Klein, E.; Farber, S.; Djerassi, I., and Arnold, P.: Hemostasis Following the Administration of Lyophilized Platelet Material in Secondary Thrombocytopenia, Proceedings of the 6th Congress of the International Society of Hematology, Boston, August, 1956, p. 339. 21. Ware, A. G., and Seegers, W. H.: Two-Stage Procedure for the Quantitative Determination of Prothrombin Concentration , Am. J. Clin. Path. 19:471-482 ( (May) ) 1949. 22. Ferguson, J. H.; Lewis, J. H., and Fresh, J. W.: The 2-Stage "Prothrombin" Assay in Study of Bleeding and Clotting Disorders , Yale J. Biol. & Med. 28:253-264 ( (Dec.-Feb.) ) 1955-1956. 23. Biggs, R., and Douglas, A. S.: The Thromboplastin Generation Test , J. Clin. Path. 6:23-29 ( (Feb.) ) 1953. 24. Lewis, J. H.; Ferguson, J. H., and Arends, T.: Hemorrhagic Disease with Circulating Inhibitors of Blood Clotting: Anti-AHF and Anti-PTC in 8 Cases , Blood 11:846-855 ( (Sept.) ) 1956. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Archives of Internal Medicine American Medical Association

Differential Diagnosis and Treatment in Hemorrhagic Disease

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Publisher
American Medical Association
Copyright
Copyright © 1957 American Medical Association. All Rights Reserved.
ISSN
0888-2479
DOI
10.1001/archinte.1957.00260070171022
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Abstract

Abstract Differential diagnosis and treatment of excessive bleeding rest upon an understanding of normal hemostatic mechanisms. Although all of the intricacies of this complicated physiological process are not fully understood, enough information is available to form a theory. The simple diagram (Fig. 1) presents the main facets of normal hemostasis. The steps probably occur simultaneously but it is convenient to consider them in four sections: vascular, tissue, platelet and plasma. Steps in Normal Hemostasis Vascular. —Intact blood vessels are obviously necessary to contain the circulating blood. In addition, blood vessels, particularly those with muscular walls, respond to mechanical (injury) or chemical stimuli (see serotonin, below) with constriction, thus reducing blood flow to an injured area. Tissue. —Normal tissues play a multiple role. First, all of the many circulating factors important in maintenance of fluidity as well as formation of the blood clot are formed in various tissue sites (Table 1). Second, References 1. Dr. Oscar Ratnoff made it possible for us to see this patient. 2. Lewis, J. H.; Tagnon, H. J.; Davidson, C. S.; Minot, G. R., and Taylor, F. H. L.: The Relation of Certain Fractions of the Plasma Globulins to the Coagulation Defect in Hemophilia , Blood 1:166-172 ( (March) ) 1946. 3. Brinkhous, K. M.: Clotting Defect in Hemophilia: Deficiency in a Plasma Factor Required for Platelet Utilization , Proc. Soc. Exper. Biol. & Med. 66:117-120 ( (Oct.) ) 1947. 4. Quick, A. J.: Studies on the Enigma of the Hemostatic Dysfunction of Hemophilia , Am. J. M. Sc. 214:272-280 ( (Sept.) ) 1947. 5. Aggeler, P. M.; White, S. G.; Glendening, M. B.; Page, E. W.; Leake, T. B., and Bates, G.: Plasma Thromboplastin Component (PTC) Deficiency: A New Disease Resembling Hemophilia , Proc. Soc. Exper. Biol. & Med. 79:692-694 ( (April) ) 1952. 6. Lewis, J. H., and Ferguson, J. H.: Hemorrhagic Diathesis Due to PTC (Plasma Thromboplastin Component) Deficiency , Proc. Soc. Exper. Biol. & Med. 82:445-448 ( (March) ) 1953. 7. Biggs, R. G.; Douglas, A. S.; Macfarlane, R. G.; Dacie, J. V.; Pitney, W. R.; Merskey, C., and O'Brien, J. R.: Christmas Disease: A Condition Previously Mistaken for Haemophilia , Brit. M. J. 2:1378-1382 ( (Dec. 27) ) 1952. 8. Rosenthal, R. L.; Dreskin, O. H., and Rosenthal, N.: New Hemophilia-like Disease Caused by Deficiency of a Third Plasma Thromboplastin Factor , Proc. Soc. Exper. Biol. & Med. 82:171-174 ( (Jan.) ) 1953. 9. Spaet, T. H.; Aggeler, P. M., and Kinsell, B. G.: A Possible Fourth Plasma Thromboplastin Component , J. Clin. Invest. 33:1095-1102 ( (Aug.) ) 1954. 10. Ratnoff, O. D., and Colopy, J. E.: A Familial Hemorrhagic Trait Associated with a Deficiency of a Clot-Promoting Fraction of Plasma , J. Clin. Invest. 34:602-613 ( (April) ) 1955. 11. Seegers, W. H. and Alkjaersig, N.: The Preparation of Prothrombin Derivatives and an Indication of Their Properties . Arch. Biochem. and Biophysics 61:1-19 ( (March) ) 1956. 12. Owren, P. A.: Prothrombin and Accessory Factors: Clinical Significance Am. J. Med. 14: 201-215 ( (Feb.) ) 1953. 13. Alexander, B.; Goldstein, R.; Landwehr, G., and Cook, C. D.: Congenital SPCA Deficiency: A Hitherto Unrecognized Coagulation Defect with Hemorrhage Rectified by Serum and Serum Fractions , J. Clin. Invest. 30:596-608 ( (June) ) 1951. 14. Owren, P. A.: Parahæmophilia: Hemorrhagic Diathesis Due to Absence of a Previously Unknown Clotting Factor , Lancet 1:446-448 ( (April 5) ) 1947. 15. Ware, A. G., and Seegers, W. H.: Plasma Accelerator Globulin: Partial Purification, Quantitative Determination, and Properties , J. Biol. Chem. 172:699-705 ( (Feb.) ) 1948. 16. Quick, A. J., and Stefanini, M.: The Concentration of the Labile Factor of the Prothrombin Complex in Human, Dog, and Rabbit Blood: Its Significance in the Determination of Prothrombin Activity , J. Lab. & Clin. Med. 33:819-826 ( (July) ) 1948. 17. Koller, F.: Symposium: What is Hemophilia? Is Hemophilia a Nosologic Entity? Blood 9:286-290 ( (March) ) 1954. 18. Lewis, J. H.; Fresh, J. W., and Ferguson, J. H.: Congenital Hypoproconvertinemia , Proc. Soc. Exper. Biol. & Med. 84:651-654 ( (Dec.) ) 1953. 19. Hougie, C., and Graham, J. B.: The Blood Clotting Role and Mode of Inheritance of the Stuart Factor: A New Clotting Factor Distinct from SPCA, Proceedings of the 6th Congress of the International Society for Blood Transfusion, Boston, September, 1956, pp. 80-81. 20. Klein, E.; Farber, S.; Djerassi, I., and Arnold, P.: Hemostasis Following the Administration of Lyophilized Platelet Material in Secondary Thrombocytopenia, Proceedings of the 6th Congress of the International Society of Hematology, Boston, August, 1956, p. 339. 21. Ware, A. G., and Seegers, W. H.: Two-Stage Procedure for the Quantitative Determination of Prothrombin Concentration , Am. J. Clin. Path. 19:471-482 ( (May) ) 1949. 22. Ferguson, J. H.; Lewis, J. H., and Fresh, J. W.: The 2-Stage "Prothrombin" Assay in Study of Bleeding and Clotting Disorders , Yale J. Biol. & Med. 28:253-264 ( (Dec.-Feb.) ) 1955-1956. 23. Biggs, R., and Douglas, A. S.: The Thromboplastin Generation Test , J. Clin. Path. 6:23-29 ( (Feb.) ) 1953. 24. Lewis, J. H.; Ferguson, J. H., and Arends, T.: Hemorrhagic Disease with Circulating Inhibitors of Blood Clotting: Anti-AHF and Anti-PTC in 8 Cases , Blood 11:846-855 ( (Sept.) ) 1956.

Journal

A.M.A. Archives of Internal MedicineAmerican Medical Association

Published: Jul 1, 1957

References