Altered Factor VIII Complexes in Patients with Acute Respiratory Insufficiency

Summary

Acute respiratory failure is an often-fatal syndrome of multiple etiologies in which altered factor VIII may be a marker of endothelial disease. 12 women with overwhelming viral pneumonia were studied with serial factor VIII antigen, procoagulant activity, and von Willebrand’s factor assays. Antigen levels were elevated (range: 86-1644%) out of proportion to procoagulant activity (range: 35-521 % by a one-stage assay), and factor VIII antigen to activity ratios were as high as 16:1. Von Willebrand’s factor was normal but correlated best with procoagulant activity. All patients had abnormal antigen patterns on crossed immunoelectrophoresis, with increases in protein of both fast and slow mobility. These changes in factor VIII correlated with the patients’ clinical courses.

• References

• 1 Bennet B, Oxnard SC, Douglas AS, Ratnoff OD. 1974; Studies on antihemophilic factor (AHF, factor VIII) during labor in normal women, in patients with premature separation of the placenta, and in a patient with von Willebrand’s disease. Journal of Laboratory and Clinical Medicine 84: 851
• 2 Blaisdell FW. 1974; Pathophysiology of the respiratory distress syndrome. Archives of Surgery 8: 44
• 3 Bone R, Francis PB, Pierce AK. 1976; Intravascular coagulation associated with the adult respiratory distress syndrome. American Journal of Medicine 61: 585
• 4 Brecher G, Cronkite EP. 1950; Morphology and enumeration of human blood platelets. Journal of Applied Physiology 3: 365
• 5 Case Records of the Massachusetts General Hospital. 1975; New England Journal of Medicine 292: 1174
• 6 Coller BS, Hirschman RJ, Gralnick HR. 1975; Studies on the factor VIII/von Willebrand factor antigen on human platelets. Thrombosis Research 6: 469
• 7 Colman RW, Robboy SJ, Minna JD. 1972; Disseminated intravascular coagulation: An approach. American Journal of Medicine 52: 679
• 8 Duckert F. 1976. Lung and coagulation. In: Junod A, Dehaller R. (eds.) Lung Metabolism. Academic Press; New York: p 251
• 9 Ellis BC, Stransky A. 1961; A quick and accurate method for the determination of fibrinogen in plasma. Journal of Laboratory and Clinical Medicine 58: 477
• 10 Green AJ, Ratnoff OD. 1974; Elevated antihemophilic factor (AHF, Factor VIII) procoagulant activity and AHF-like antigen in alcoholic cirrhosis of the liver. Journal of laboratory and Clinical Medicine 83: 189
• 11 Hargreaves LN, Todd AS. 1976; High levels of factor VIII-related antigen in cadaveric blood. Thrombosis Research 9: 301
• 12 Heiden D, Mielke Jr CH, Rodvien R, Hill JD. 1975; Platelets, hemostasis, and thromboembolism during treatment of acute respiratory insufficiency with extracorporeal membrane oxagenation. Journal of Thoracic Cardiovascular Surgery 70: 644
• 13 Hers JF, Mulder J. 1961; Broad aspects of the pathology and pathogenesis of human influenza. American Review of Respiratory Diseases 83 (02) 84
• 14 Hill JD, Delaval MR, Mielke Jr CH, Bramson ML, Gerbode F. 1972; Clinical prolonged extracorporeal circulation for respiratory insufficiency: Hematological effects. Transactions of the American Society of Artificial Organs XVII: 546
• 15 Hill JD, Ratliff JL, Panot JC, Lamy M, Fallat RJ, Koeniger E, Yaeger EM, Whitmer G. 1976; Pulmonary pathology in acute respiratory insufficiency: Lung biopsy as a diagnostic tool. Journal of Thoracic and Cardiovascular Surgery 71: 64
• 16 Jaffe E. 1977; Endothelial cells and the biology of factor VIII. New England Journal of Medicine 296: 377
• 17 Laurell CB. 1965; Antigen antibody crossed electrophoresis. Analytical Biochemistry 10: 358
• 18 Lian EC, Nunez RL, Harkness DR. 1976; In vivo and in vitro effects of thrombin and plasmin on human factor VIII (AHF). American Journal of Haematology 1: 481
• 19 Maisonneuve P, Sultan Y. 1977; Modification of factor VIII complex properties in patients with liver disease. Journal of Clinical Pathology 30: 221
• 20 Mason R, Sharp D, Chuang HY K, Mohammed SF. 1977; The endothelium. Archives of Pathology and Laboratory Medicine 101: 61
• 21 Mayer G, Kalogjera V, Witz JP, Fongio J, Waitz B. 1974; Etude die taux du facteur VIII dans les vaisseaux profonds de 1’organisme. Blut 28: 264
• 22 Mckee PA, Andersen JC, Switzer ME. 1975; Molecular structural studies of human factor VIII. Annals of the New York Academy of Sciences 240: 8
• 23 Merskey C, Johnson AJ, Lalezari P. 1972; Increase in fibrinogen and fibrin-related antigen in human serum due to in vitro lysis of fibrin by thrombin. Journal of Clinical Investigation 51: 903
• 24 Mielke Jr CH, Rodvien R, May N, Hill JD. 1976 Coagulopathy of acute respiratory insufficiency. Abstract XVI International Congress of Hematology, Kyoto, Japan
• 25 Moncada S, Higgs EA, Vane JR. 1977; Human arterial and venous tissues generate prostacyclin (prostaglandin X), a potent inhibitor of platelet aggregation. Lancet 1: 18
• 26 Pasquini R, Hershgold EJ. 1973; Effects of plasmin on human factor VIII (AHF). Blood 41: 105
• 27 Petty TL, Ashbaugh DG. 1971; The adult respiratory distress syndrome. Chest 60: 233
• 28 Proctor RR, Rapaport SI. 1961; The partial thromboplastin time with kaolin: A simple screening test for first stage plasma clotting factor deficiencies. American Journal of Clinical Pathology 36: 212
• 29 Quick AJ. 1957. Hemorrhagic Diseases. Lea & Febiger Publishers; Philadelphia: p 377
• 30 Risberg B, Peterson HI, Zettergren L. 1975; Fibrinolysis of the lung: An experimental study on the localization of plasminogen activator in the rat lung. Microvascular Research 9: 222
• 31 Robboy SJ, Colman RW, Minna JD. 1974; Pathology of disseminated intravascular coagulation (DIC). Human Pathology 3: 327
• 32 Saba SR, Mason RG. 1974; Studies of an activity from endothelial cells that inhibits platelet aggregation, serotonin release, and clot retraction. Thrombosis Research 5: 747
• 33 Simone JV, Vanderheiden J, Abildgaard CF. 1967; A semiautomatic one stage factor VIII assay with a commercially prepared standard. Journal of Laboratory and Clinical Medicine 69: 708
• 34 Stibbe J. 1977; Effect of exercise on factor VIII complex: Proportional increase of ristocetin cofactor (von Willebrand factor) and factor VIII-antigen, but disproportional increase of factor VIII-AHF. Thrombosis Research 10: 163
• 35 Strum JM, Junod AF. 1972; Radioautographic demonstration of 5-hydroxytryptamine-³H uptake by pulmonary endothelial cells. Journal of Cell Biology 54: 456
• 36 Sultan Y, Simeon J, Caen JP. 1976; Electrophoretic heterogeneity of normal factor VIII/von Willebrand protein, and abnormal electrophoretic mobility in patients with von Willebrand’s disease. Journal of Laboratory and Clinical Medicine 87: 185
• 37 Weiss HJ, Hoyer LW. 1973; a Quantitative assay of a plasma factor deficient in von Willebrand’s disease that is necessary for platelet aggregation. Journal of Clinical Investigation 52: 2708
• 38 Weiss HJ, Hoyer LW. 1973; b Von Willebrand factor: Disassociation from antihemophilic factor procoagulant activity. Science 182: 1149
• 39 Zimmerman TS, Edgington TS. 1974; Factor VIII coagulant activity and factor VIII-like antigen: Independent molecular entities. Journal of Experimental Medicine 138: 1015
• 40 Zimmerman TS, Hoyer LW, Dicksen L, Edgington TS. 1975; a Determination of the von Willebrand’s disease antigen (factor VIII-related antigen) in plasma by quantitative immunoelectrophoresis. Journal of Laboratory and Clinical Medicine 86: 152
• 41 Zimmerman TS, Roberts J, Edgington TS. 1975; b Factor VIII-related antigen: Multiple molecular forms in human plasma. Proceedings of the National Academy of Sciences 75: 5121
• 42 Zimmerman TS. 1976 Personal communication.