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The Hemophilioid States: The Practical Application of Present-Day Concepts of Blood Coagulation to Diagnosis and Treatment

The Hemophilioid States: The Practical Application of Present-Day Concepts of Blood Coagulation... Abstract During the past decade there has developed an aura of growing alarm and bewilderment at the progressive complexity of blood coagulation concepts. In the face of this concern, it would seem desirable to review briefly the nature of these concepts and to discuss their practical applications in general medical practice, with particular emphasis on the hemophilioid states. The hemorrhagic disorders may be divided into three etiologic groups. Etiologic Groups of Hemorrhagic Disorders Congenital Hemophilioid Disorders1-21 (Table 1).These are ordinarily understood to be hemorrhagic disorders that clinically resemble hemophilia resulting from a genetically determined deficiency or inactivity of a factor in plasma which is necessary for an effective prothrombin-converting and fibrin-forming mechanism.1In this communication hemophilia will be included under the heading of a congenital hemophilioid state, although this is not strictly in accord with the common usage of this term. Acquired hemorrhagic states22-35 closely resembling hemophilia References 1. Plasma treated with aluminum hydroxide or barium sulfate to adsorb prothrombin, PTC, Stuart factor, and Factor VII. 2. It has been shown that Factor VII, although required in the extrinsic system, is not necessary for the formation of thromboplastin in the intrinsic system. 3. The platelet factor contributing to the generation of intrinsic thromboplastin. 4. The screening tests for thromboplastin generation do not reflect a deficiency of Platelet Factor 3, since a platelet substitute, cephalin, is added to the test plasma. 5. A recent experience of one of us (C. H.), in which over 2 liters of plasma given over a period of 24 hours failed to achieve a hemostatic level in a moderately severe case of Christmas disease, suggests that considerably larger doses of plasma may be required in this condition. 6. John B. Graham (personal communication) 7. Brinkhous, K. M.: Hemophilia and Hemophilioid Diseases , Chapel Hill, N. C., The University of North Carolina Press, 1957. 8. Brinkhous, K. M.; Langdell, R. D., and Wagner, R. H.: Hemostatic Disorders: Hemophilia and the Hemophilioid Diseases , Ann. Rev. Med. 9:159, 1958.Crossref 9. 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, 1952. 10. Biggs, R.; 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, 1952. 11. Schulman, I., and Smith, C. H.: Hemorrhagic Disease in an Infant Due to Deficiency of a Previously Underscribed Clotting Factor , Blood 7:794, 1952. 12. Owren, P. A.: Coagulation of Blood: Investigations on a New Clotting Factor , Acta med. scandinav. (Supp. 194) , 1947. 13. Quick, A. J.: On the Constitution of Prothrombin , Am. J. Physiol. 140:212, 1943. 14. Fantl, P., and Nance, M. H.: Acceleration of Thrombin Formation by a Plasma Component , Nature 158:708, 1946. 15. Ware, A. G., and Seegers, W. H.: Plasma Accelerator Globulin: Partial Purification, Quantitative Determination and Properties , J. Biol. Chem. 172:699, 1948. 16. 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, 1955. 17. 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, 1953. 18. Bernard, J., and Soulier, J. P.: Sur une nouvelle Variété de dystrophie thrombocytaire hémorragipare congénitale , Semaine hôp. Paris 24: 3217, 1948. 19. Hougie, C.; Barrow, E. M., and Graham, J. B.: Stuart Clotting Defect: I. Segregation of an Hereditary Hemorrhagic State from the Heterogeneous Group Heretofore Called "Stable Factor" (SPCA, Proconvertin, Factor VII) Deficiency , J. Clin. Invest. 36:485, 1957. 20. Telfer, T. P.; Denson, K. W., and Wright, D. R.: A "New" Coagulation Defect , Brit. J. Haemat. 2:308, 1956. 21. Schultze, H. E., and Schwick, G.: Über eine neuartige "hämorrhagische" Diathese , Blut 3:233, 1957. 22. Bachmann, F.; Duckert, F.; Geiger, M.; Baer, P., and Koller, F.: Differentiation of the Factor VII Complex: Studies on the Stuart-Prower Factor , Thromb. et. diath. haem. 8:169, 1957. 23. Alexander, B.; Goldstein, R.; Landwehr, G., and Cook, C. D.: Congenital SPCA Deficiency: A Hitherto Unrecognized Coagulation Defect with Hemorrhage Rectified by Serum Fractions , J. Clin. Invest. 30:596, 1951. 24. Koller, F.; Loeliger, A., and Duckert, F.: Experiments on a New Clotting Factor (Factor VII) , Acta haemat. 6:1, 1951. 25. Owren, P. A.: Proconvertin, the New Clotting Factor , Scandinav. J. Clin. & Lab. Invest. 3:168, 1951. 26. Quick, A. J.; Pisciotta, A. V., and Hussey, C. V.: Congenital Hypoprothombinemic States , A. M. A. Arch. Int. Med. 95:2, 1955. 27. Rabe, F., and Salomon, E.: Über Faserstoffmangel im Blute bei einem Falle von Hämophilie , Deutsches Arch. klin. Med. 132:240, 1920. 28. Hougie, C.: Circulating Anticoagulants , Brit. M. Bull. 11:16, 1955. 29. 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, 1956. 30. Hougie, C., and Fearnley, M. E.: The Nature and Action of Circulating Anti-Coagulants , Acta haemat. 12:1, 1954.Crossref 31. Conley, C. L., and Hartmann, R. C.: A Hemorrhagic Disorder Caused by Circulating Anticoagulant in Patients with Disseminated Lupus Erythematosis , J. Clin. Invest. 31:621, 1952. 32. Nilsson, I. M.; Skanse, B., and Gydell, K.: Circulating Anticoagulant After Pregnancy and Its Response to ACTH , Acta haemat. 19:40, 1958.Crossref 33. Ferguson, J. H.; Johnson, C. L., Jr., and Howell, D. A.: A Circulating Inhibitor (Anti-AcG) Specific for the Labile Factor-V of the Blood Clotting Mechanism , Blood 13:382, 1958. 34. Waddell, W. W., Jr., and Guerry, D., III: The Role of Vitamin K in the Etiology, Prevention, and Treatment of Hemorrhage in the Newborn Infant, Part II , J. Pediat. 15:802, 1939.Crossref 35. Dieckmann, W. J.: Blood Chemistry and Renal Function in Abruptio Placentae , Am. J. Obst. & Gynec. 31:734, 1937. 36. Astrup, T.: Fibrinolysis in the Organism , Blood 11:781, 1956. 37. Sherry S., and Alkjaersig, N.: Studies on the Fibrinolytic Enzyme of Human Plasma , Thromb. et diath. haem. 1:364, 1957. 38. Tagnon, H. J.; Whitmore, W. F., Jr., and Shulman, N. R.: Fibrinolysis in Metastatic Cancer of the Prostate , Cancer 5:9, 1952. 39. Ratnoff, O. D.: Plasma Studies on Proteolytic Enzymes in Human Plasma: IV. The Rate of Lysis of Plasma Clots in Normal and Diseased Individuals with Particular Reference to Hepatic Disease , Bull. Johns Hopkins Hosp. 84:29, 1949. 40. Lewis, J. H.; Zucker, M. B., and Ferguson, J. H.: Bleeding Tendency in Uremia , Blood 11:1073, 1956. 41. Lewis, J. H.; Burchenal, J. H.; Ellison, R. R.; Ferguson, J. H.; Palmer, J. H.; Murphy, M. L., and Zucker, M. B.: Studies of Hemostatic Mechanisms in Leukemia and Thrombocytopenia , Am. J. Clin. Path. 28:433, 1957. 42. Harrington, W. J.: The Purpuras , DM (July) , 1957. 43. Spaet, T. H.; Bauer, S., and Melamed S.: Hemorrhagic Thrombocythemia: A Blood Coagulation Disorder , A. M. A. Arch. Int. Med. 98:377, 1956. 44. Buchanan, J. C., and Leavell, B. S.: Pseudohemophilia: Report of 12 New Cases and Statistical Review of Previously Reported Cases , Ann. Int. Med. 44:214, 1956. 45. Alexander, B., and Goldstein, R.: Dual Hemostatic Defect in Pseudohemophilia , J. Clin. Invest. 32:551, 1953. 46. Singer, K., and Ramot, B.: Pseudohemophilia Type B , A. M. A. Arch. Int. Med. 97:715, 1956. 47. Shulman, I.; Smith, C. H.; Erlandson, M., and Fort, E.: Vascular Hemophilia: A Familial Hemorrhagic Disease in Males and Females Characterized by Combined Antihemophilic Globulin Deficiency and Vascular Abnormality , A. M. A. Am. J. Dis. Child. 90:526, 1955. 48. Nilsson, I. M.; Blombäck, M.; Jorpes, E.; Blombäck, B., and Johansson, S.: v. Willebrand's Disease and Its Correction with Human Plasma Fraction 1-0 , Acta med. scandinav. 159:179, 1957. 49. von Willebrand, E. A., and Jürgens, R.: Über eine neue Bluterkrankheit: Die konstitutionelle Thrombopathie , Klin. Wchnschr. 12:414, 1933. 50. Glanzmann, E.: Hereditäre hämorrhagische Thrombasthenie , Jahrb. Kinderh. 88:113, 1918. 51. Macfarlane, R. G.: Blood Coagulation with Particular Reference to the Early Stages , Physiol. Rev. 36:479, 1956. 52. Biggs, R., and Macfarlane, R. G.: Human Blood Coagulation and Its Disorders , Ed. 2, Springfield, Ill., Charles C Thomas, Publisher, 1957. 53. Duckert, F.; Koller, F., and Matter, M.: Purification and Physiological Properties of Factor VII from Plasma and Serum: Separation from Prothrombin , Proc. Soc. Exper. Biol. & Med. 82: 259, 1953. 54. Goldstein, R., and Alexander, B.: Further Studies on Proconvertin Deficiency and the Role of Proconvertin , in Hemophilia and Hemophilioid Diseases , edited by K. M. Brinkhous, Chapel Hill, N. C., The University of North Carolina Press, 1957, p. 93. 55. Quick, A. J.: Hemorrhagic Diseases , Philadelphia, Lea & Febiger, 1957. 56. Diggs, L. W.: Diagnosis of Hemorrhagic Diseases: Evaluation of Procedures; I. History, Physical Examination , California Med. 87:361, 1957. 57. Vitamin C Requirement of Human Adults , Medical Research Council, Special Report Series 280, London, Her Majesty's Stationery Office, 1953. 58. Duke, W. W.: The Relation of Blood Platelets to Hemorrhagic Disease , J. A. M. A. 55:1185, 1910. 59. Ivy, A. C.; Nelson, D., and Bucher, G.: The Standardization of Certain Factors in the Cutaneous "Venostasis" Bleeding Time Technique , J. Lab. & Clin. Med. 26:1812, 1940. 60. Brecher, G., and Cronkite, E. P.; Morphology and Enumeration of Human Blood Platelets , J. Appl. Physiol. 3:365, 1950. 61. Biggs, R., and Douglas, A. S.: The Thromboplastin Generation Test , J. Clin. Path. 6:23, 1953. 62. Bell, W. N., and Alton, H. G.: Brain Extract as Substitute for Platelet Suspensions in Thromboplastin Generation Test , Nature, London 174:880, 1954. 63. Ingram, G. I.: Observations in a Case of Multiple Haemostatic Defect , Brit. J. Haemat. 2:180, 1956. 64. Wagner, R. H.; Graham, G. B.; Penick, G. D., and Brinkhous, K. M.: Estimation of Prothrombin by the 2-Stage Method , in the Coagulation of Blood: Methods of Study , edited by L. M. Tocantins, New York, Grune & Stratton, Inc., 1955, p. 105. 65. Bergsagel, D. E., and Hougie, C.: Intermediate Stages in the Formation of Blood Thromboplastin , Brit. J. Haemat. 2:113, 1956. 66. Hougie, C.: The Role of Factor V in the Formation of Blood Thromboplastin , J. Lab. & Clin. Med. 50:61, 1957. 67. Langdell, R. D.; Wagner, R. H., and Brinkhous, K. M.: Effect of Antihemophilic Factor on One-Stage Clotting Tests , J. Lab. & Clin. Med. 41:637, 1953. 68. Rodman, N. F., Jr.; Barrow, E. M., and Graham, J. B.: Diagnosis and Control of the Hemophilioid States with the Partial Thromboplastin Time (PTT) Test , Am. J. Clin. Path. 29:525, 1958. 69. Hicks, N. D., and Pitney, W. R.: A Rapid Screening Test for Disorders of Thromboplastin Generation , Brit. J. Haemat. 3:227, 1957. 70. Langdell, R. D.: Transfusion Therapy in Hemophilia: Hemophilia and Hemophilioid Diseases , edited by K. M. Brinkhous, Chapel Hill, N. C., The University of North Carolina Press, 1957, pp. 189-193. 71. Biggs, R.: Recent Advances in the Diagnosis and Treatment of Haemophilia , Proc. Roy. Soc. Med. 50:645, 1957. 72. Pitney, W. R.: The Assay of Antihaemophilic Globulin (AHG) in Plasma , Brit. J. Haemat. 2:250, 1956. 73. Brinkhous, K. M.; Penick, G. D.; Langdell, R. D.; Wagner, R. H., and Graham, J. B.: Physiologic Basis of Transfusion Therapy in Hemophilia , A. M. A. Arch. Path. 61:6, 1956. 74. Penick, G. D., and Brinkhous, K. M.: Relative Stability of Plasma Anti-Hemophilic Factor (AHF) Under Different Conditions of Storage , Am. J. M. Sc. , 232:434, 1956. 75. Biggs, R.: Assay of Antihaemophilic Globulin in Treatment of Haemophilic Patients , Lancet 2:311, 1957. 76. Wagner, R. H.; Langdell, R. D.; Richardson, B. A.; Farrell, R. A., and Brinkhous, K. M.: Antihemophilic Factor (AHF): Plasma Levels After Administration of AHF Preparations to Hemophilic Dogs , Proc. Soc. Exper. Biol. & Med. 96:152, 1957. 77. Sharp, A. A., and Bidwell, E.: The Toxicity and Fate of Injected Animal Plasma , Lancet 2: 359, 1957. 78. Bidwell, E.: The Purification of Antihaemophilic Globulin from Animal Plasma , Brit. J. Haemat. 1:386, 1955. 79. Macfarlane, R. G.; Mallam, P. C.; Witts, L. J.; Bidwell, E.; Biggs, R.; Fraenkel, G. J.; Honey, G. E., and Taylor, K. B.: Surgery in Haemophilia: The Use of Animal Antihaemophilic Globulin and Human Plasma in 13 Cases , Lancet 2:251, 1957. 80. Kekwick, R. A., and Wolf, P.: A Concentrate of Human Antihaemophilic Factor: Its Use in 6 Cases of Haemophilia , Lancet 1:647, 1957. 81. Aggeler, P. M.: Plasma Thromboplastin Component (PTC) Deficiency , in Hemophilia and Hemophilioid Diseases , edited by K. M. Brinkhous, Chapel Hill, N. C., The University of North Carolina Press, 1957, pp. 111-115. 82. Jackson, D. P.; Krevans, J. R., and Conley, C. L.: Mechanism of the Thrombocytopenia That Follows Multiple Whole Blood Transfusions , Tr. A. Am. Physicians 69:155, 1956. 83. Orr, J. A., and Douglas, A. S.: Dental Extraction in Haemophilia and Christmas Disease. Brit. M. J. 1:1035, 1957. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Archives of Internal Medicine American Medical Association

The Hemophilioid States: The Practical Application of Present-Day Concepts of Blood Coagulation to Diagnosis and Treatment

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References (85)

Publisher
American Medical Association
Copyright
Copyright © 1959 American Medical Association. All Rights Reserved.
ISSN
0888-2479
DOI
10.1001/archinte.1959.00270020067008
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Abstract

Abstract During the past decade there has developed an aura of growing alarm and bewilderment at the progressive complexity of blood coagulation concepts. In the face of this concern, it would seem desirable to review briefly the nature of these concepts and to discuss their practical applications in general medical practice, with particular emphasis on the hemophilioid states. The hemorrhagic disorders may be divided into three etiologic groups. Etiologic Groups of Hemorrhagic Disorders Congenital Hemophilioid Disorders1-21 (Table 1).These are ordinarily understood to be hemorrhagic disorders that clinically resemble hemophilia resulting from a genetically determined deficiency or inactivity of a factor in plasma which is necessary for an effective prothrombin-converting and fibrin-forming mechanism.1In this communication hemophilia will be included under the heading of a congenital hemophilioid state, although this is not strictly in accord with the common usage of this term. Acquired hemorrhagic states22-35 closely resembling hemophilia References 1. Plasma treated with aluminum hydroxide or barium sulfate to adsorb prothrombin, PTC, Stuart factor, and Factor VII. 2. It has been shown that Factor VII, although required in the extrinsic system, is not necessary for the formation of thromboplastin in the intrinsic system. 3. The platelet factor contributing to the generation of intrinsic thromboplastin. 4. The screening tests for thromboplastin generation do not reflect a deficiency of Platelet Factor 3, since a platelet substitute, cephalin, is added to the test plasma. 5. A recent experience of one of us (C. H.), in which over 2 liters of plasma given over a period of 24 hours failed to achieve a hemostatic level in a moderately severe case of Christmas disease, suggests that considerably larger doses of plasma may be required in this condition. 6. John B. Graham (personal communication) 7. Brinkhous, K. M.: Hemophilia and Hemophilioid Diseases , Chapel Hill, N. C., The University of North Carolina Press, 1957. 8. Brinkhous, K. M.; Langdell, R. D., and Wagner, R. H.: Hemostatic Disorders: Hemophilia and the Hemophilioid Diseases , Ann. Rev. Med. 9:159, 1958.Crossref 9. 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, 1952. 10. Biggs, R.; 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, 1952. 11. Schulman, I., and Smith, C. H.: Hemorrhagic Disease in an Infant Due to Deficiency of a Previously Underscribed Clotting Factor , Blood 7:794, 1952. 12. Owren, P. A.: Coagulation of Blood: Investigations on a New Clotting Factor , Acta med. scandinav. (Supp. 194) , 1947. 13. Quick, A. J.: On the Constitution of Prothrombin , Am. J. Physiol. 140:212, 1943. 14. Fantl, P., and Nance, M. H.: Acceleration of Thrombin Formation by a Plasma Component , Nature 158:708, 1946. 15. Ware, A. G., and Seegers, W. H.: Plasma Accelerator Globulin: Partial Purification, Quantitative Determination and Properties , J. Biol. Chem. 172:699, 1948. 16. 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, 1955. 17. 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, 1953. 18. Bernard, J., and Soulier, J. P.: Sur une nouvelle Variété de dystrophie thrombocytaire hémorragipare congénitale , Semaine hôp. Paris 24: 3217, 1948. 19. Hougie, C.; Barrow, E. M., and Graham, J. B.: Stuart Clotting Defect: I. Segregation of an Hereditary Hemorrhagic State from the Heterogeneous Group Heretofore Called "Stable Factor" (SPCA, Proconvertin, Factor VII) Deficiency , J. Clin. Invest. 36:485, 1957. 20. Telfer, T. P.; Denson, K. W., and Wright, D. R.: A "New" Coagulation Defect , Brit. J. Haemat. 2:308, 1956. 21. Schultze, H. E., and Schwick, G.: Über eine neuartige "hämorrhagische" Diathese , Blut 3:233, 1957. 22. Bachmann, F.; Duckert, F.; Geiger, M.; Baer, P., and Koller, F.: Differentiation of the Factor VII Complex: Studies on the Stuart-Prower Factor , Thromb. et. diath. haem. 8:169, 1957. 23. Alexander, B.; Goldstein, R.; Landwehr, G., and Cook, C. D.: Congenital SPCA Deficiency: A Hitherto Unrecognized Coagulation Defect with Hemorrhage Rectified by Serum Fractions , J. Clin. Invest. 30:596, 1951. 24. Koller, F.; Loeliger, A., and Duckert, F.: Experiments on a New Clotting Factor (Factor VII) , Acta haemat. 6:1, 1951. 25. Owren, P. A.: Proconvertin, the New Clotting Factor , Scandinav. J. Clin. & Lab. Invest. 3:168, 1951. 26. Quick, A. J.; Pisciotta, A. V., and Hussey, C. V.: Congenital Hypoprothombinemic States , A. M. A. Arch. Int. Med. 95:2, 1955. 27. Rabe, F., and Salomon, E.: Über Faserstoffmangel im Blute bei einem Falle von Hämophilie , Deutsches Arch. klin. Med. 132:240, 1920. 28. Hougie, C.: Circulating Anticoagulants , Brit. M. Bull. 11:16, 1955. 29. 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, 1956. 30. Hougie, C., and Fearnley, M. E.: The Nature and Action of Circulating Anti-Coagulants , Acta haemat. 12:1, 1954.Crossref 31. Conley, C. L., and Hartmann, R. C.: A Hemorrhagic Disorder Caused by Circulating Anticoagulant in Patients with Disseminated Lupus Erythematosis , J. Clin. Invest. 31:621, 1952. 32. Nilsson, I. M.; Skanse, B., and Gydell, K.: Circulating Anticoagulant After Pregnancy and Its Response to ACTH , Acta haemat. 19:40, 1958.Crossref 33. Ferguson, J. H.; Johnson, C. L., Jr., and Howell, D. A.: A Circulating Inhibitor (Anti-AcG) Specific for the Labile Factor-V of the Blood Clotting Mechanism , Blood 13:382, 1958. 34. Waddell, W. W., Jr., and Guerry, D., III: The Role of Vitamin K in the Etiology, Prevention, and Treatment of Hemorrhage in the Newborn Infant, Part II , J. Pediat. 15:802, 1939.Crossref 35. Dieckmann, W. J.: Blood Chemistry and Renal Function in Abruptio Placentae , Am. J. Obst. & Gynec. 31:734, 1937. 36. Astrup, T.: Fibrinolysis in the Organism , Blood 11:781, 1956. 37. Sherry S., and Alkjaersig, N.: Studies on the Fibrinolytic Enzyme of Human Plasma , Thromb. et diath. haem. 1:364, 1957. 38. Tagnon, H. J.; Whitmore, W. F., Jr., and Shulman, N. R.: Fibrinolysis in Metastatic Cancer of the Prostate , Cancer 5:9, 1952. 39. Ratnoff, O. D.: Plasma Studies on Proteolytic Enzymes in Human Plasma: IV. The Rate of Lysis of Plasma Clots in Normal and Diseased Individuals with Particular Reference to Hepatic Disease , Bull. Johns Hopkins Hosp. 84:29, 1949. 40. Lewis, J. H.; Zucker, M. B., and Ferguson, J. H.: Bleeding Tendency in Uremia , Blood 11:1073, 1956. 41. Lewis, J. H.; Burchenal, J. H.; Ellison, R. R.; Ferguson, J. H.; Palmer, J. H.; Murphy, M. L., and Zucker, M. B.: Studies of Hemostatic Mechanisms in Leukemia and Thrombocytopenia , Am. J. Clin. Path. 28:433, 1957. 42. Harrington, W. J.: The Purpuras , DM (July) , 1957. 43. Spaet, T. H.; Bauer, S., and Melamed S.: Hemorrhagic Thrombocythemia: A Blood Coagulation Disorder , A. M. A. Arch. Int. Med. 98:377, 1956. 44. Buchanan, J. C., and Leavell, B. S.: Pseudohemophilia: Report of 12 New Cases and Statistical Review of Previously Reported Cases , Ann. Int. Med. 44:214, 1956. 45. Alexander, B., and Goldstein, R.: Dual Hemostatic Defect in Pseudohemophilia , J. Clin. Invest. 32:551, 1953. 46. Singer, K., and Ramot, B.: Pseudohemophilia Type B , A. M. A. Arch. Int. Med. 97:715, 1956. 47. Shulman, I.; Smith, C. H.; Erlandson, M., and Fort, E.: Vascular Hemophilia: A Familial Hemorrhagic Disease in Males and Females Characterized by Combined Antihemophilic Globulin Deficiency and Vascular Abnormality , A. M. A. Am. J. Dis. Child. 90:526, 1955. 48. Nilsson, I. M.; Blombäck, M.; Jorpes, E.; Blombäck, B., and Johansson, S.: v. Willebrand's Disease and Its Correction with Human Plasma Fraction 1-0 , Acta med. scandinav. 159:179, 1957. 49. von Willebrand, E. A., and Jürgens, R.: Über eine neue Bluterkrankheit: Die konstitutionelle Thrombopathie , Klin. Wchnschr. 12:414, 1933. 50. Glanzmann, E.: Hereditäre hämorrhagische Thrombasthenie , Jahrb. Kinderh. 88:113, 1918. 51. Macfarlane, R. G.: Blood Coagulation with Particular Reference to the Early Stages , Physiol. Rev. 36:479, 1956. 52. Biggs, R., and Macfarlane, R. G.: Human Blood Coagulation and Its Disorders , Ed. 2, Springfield, Ill., Charles C Thomas, Publisher, 1957. 53. Duckert, F.; Koller, F., and Matter, M.: Purification and Physiological Properties of Factor VII from Plasma and Serum: Separation from Prothrombin , Proc. Soc. Exper. Biol. & Med. 82: 259, 1953. 54. Goldstein, R., and Alexander, B.: Further Studies on Proconvertin Deficiency and the Role of Proconvertin , in Hemophilia and Hemophilioid Diseases , edited by K. M. Brinkhous, Chapel Hill, N. C., The University of North Carolina Press, 1957, p. 93. 55. Quick, A. J.: Hemorrhagic Diseases , Philadelphia, Lea & Febiger, 1957. 56. Diggs, L. W.: Diagnosis of Hemorrhagic Diseases: Evaluation of Procedures; I. History, Physical Examination , California Med. 87:361, 1957. 57. Vitamin C Requirement of Human Adults , Medical Research Council, Special Report Series 280, London, Her Majesty's Stationery Office, 1953. 58. Duke, W. W.: The Relation of Blood Platelets to Hemorrhagic Disease , J. A. M. A. 55:1185, 1910. 59. Ivy, A. C.; Nelson, D., and Bucher, G.: The Standardization of Certain Factors in the Cutaneous "Venostasis" Bleeding Time Technique , J. Lab. & Clin. Med. 26:1812, 1940. 60. Brecher, G., and Cronkite, E. P.; Morphology and Enumeration of Human Blood Platelets , J. Appl. Physiol. 3:365, 1950. 61. Biggs, R., and Douglas, A. S.: The Thromboplastin Generation Test , J. Clin. Path. 6:23, 1953. 62. Bell, W. N., and Alton, H. G.: Brain Extract as Substitute for Platelet Suspensions in Thromboplastin Generation Test , Nature, London 174:880, 1954. 63. Ingram, G. I.: Observations in a Case of Multiple Haemostatic Defect , Brit. J. Haemat. 2:180, 1956. 64. Wagner, R. H.; Graham, G. B.; Penick, G. D., and Brinkhous, K. M.: Estimation of Prothrombin by the 2-Stage Method , in the Coagulation of Blood: Methods of Study , edited by L. M. Tocantins, New York, Grune & Stratton, Inc., 1955, p. 105. 65. Bergsagel, D. E., and Hougie, C.: Intermediate Stages in the Formation of Blood Thromboplastin , Brit. J. Haemat. 2:113, 1956. 66. Hougie, C.: The Role of Factor V in the Formation of Blood Thromboplastin , J. Lab. & Clin. Med. 50:61, 1957. 67. Langdell, R. D.; Wagner, R. H., and Brinkhous, K. M.: Effect of Antihemophilic Factor on One-Stage Clotting Tests , J. Lab. & Clin. Med. 41:637, 1953. 68. Rodman, N. F., Jr.; Barrow, E. M., and Graham, J. B.: Diagnosis and Control of the Hemophilioid States with the Partial Thromboplastin Time (PTT) Test , Am. J. Clin. Path. 29:525, 1958. 69. Hicks, N. D., and Pitney, W. R.: A Rapid Screening Test for Disorders of Thromboplastin Generation , Brit. J. Haemat. 3:227, 1957. 70. Langdell, R. D.: Transfusion Therapy in Hemophilia: Hemophilia and Hemophilioid Diseases , edited by K. M. Brinkhous, Chapel Hill, N. C., The University of North Carolina Press, 1957, pp. 189-193. 71. 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Journal

A.M.A. Archives of Internal Medicine – American Medical Association

Published: Feb 1, 1959

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