Summary
Fifty-tow patients with sickle-cell (SC) disease (48 with SC-beta-thalassaemia and 4 with homozygous SC-anaemia) were studied as regards blood coagulation and fibrinolysis. It was found that the thrombin and the reptilase times of the patients' plasma were significantly shorter than normal. The mean values of platelet count, fibrinogen level and factor VIII activity of patients with SC disease were higher than normal; however, in the group of patients transfused, with less than 50% haemoglobin S (HbS), the fibrinogen level and the factor VIII activity were significantly lower compared to the other patients. Antithrombin-III (At-III) activity was normal in all. The fibrinolytic activity was normal in patients with asymptomatic SC disease, but reduced in patients on painful crises. Plasminogen and fibrinogen/fibrin degradation product (FDP) levels were normal in all patients. Two patients on painful crises with complications had additional abnormal findings, namely prolonged prothrombin time, reduced At-III level and elevated FDP.
Zusammenfassung
Bei 52 Patienten mit Sichelzell-Anämie (SC) (48 mit SC-β-Thalassämie und 4 mit homozygoter SC-Anämie) wurden Blutgerinnung und Fibrinolyse untersucht. Es wurde festgestellt, daß die Thrombin-und Reptilasezeiten bedeutend kürzer waren als normal. Die Durchschnittswerte von Thrombozytenzahl, Fibrinogenwert und Faktor-VIII-Aktivität der Patienten mit SC-Anämie waren höher als normal; bei transfundierten Patienten mit weniger als 50% HbS waren Fibrinogenwert und Faktor-VIII-Aktivität bedeutend niedriger als bei den übrigen. Die fibrinolytische Aktivität war bei Patienten mit asymptomatischer SC-Krankheit normal, bei Patienten mit schmerzhaften Krisen erniedrigt. Antithrombin III, Plasminogen und Fibrinogenspaltprodukte waren in allen Fällen normal. Nur zwei Patienten mit schmerzhaften Krisen und Komplikationen hatten zusätzliche abnormale Befunde, wie verlängerte Prothrombinzeit, verminderter Antithrombin-III-Wert und Vermehrung von Fibrinogen-Spaltprodukten.
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References
Abildgaard C. F., Simone J. V. & Schulman I.: Factor-VIII (antihaemophilic factor) activity in sickle-cell anaemia. Brit. J. Haemat.13, 19 (1967).
Abildgaard U., Gravem K. & Godal H. C.: Assay of progressive antithrombin in plasma. Thromb. Diath. haemorrh.24, 224 (1970).
Alkjaersig N., Fletcher A. P. & Sherry S.: The mechanism of clot resolution by plasmin. J. clin. Invest.38, 1086 (1959).
Amris C. J. & Hansen N. E.: Coagulation and fibrinolytic studies in paroxysmal nocturnal hemoglobinuria. Acta med. Scand.184, 551 (1968).
Astrup T. & Mullertz S.: The fibrin plate method for estimating fibrinolytic activity. Arch. Biochem.40, 346 (1952).
Biggs R. & MacFarlane R. G.: Human Blood Coagulation and its Disorders (3rd ed.) p. 370. Blackwell Scientific Publications, Oxford, 1962.
Brecher G. & Cronkite C. P.: Morphology and enumeration of human blood platelets. J. appl. Physiol.3, 365 (1950).
Dacie J. V. & Lewis S. M.: Practical Haematology (4th ed.). Churchill, London 1968.
Diggs L. W.: Sickle cell crises. Amer. J. clin. Path.44, 1 (1965).
Donegan C. C., Jr., MacIlwaine W. A. & Leavell B. S.: Haematologic studies on patients with sickle cell anaemia following multiple transfusions. Amer. J. Med.17, 29 (1954).
Egeberg O: Blood coagulation and intravascular haemolysis. Scand J. clin. lab. Invest.14, 217 (1962).
Green D., Kwaan H. C. & Ruiz G.: Impaired fibrinolysis in sickle cell disease. Relation to crisis and infection. Thromb. Diath. haemorrh.24, 10 (1970).
Hardisty R. M. & MacPherson J. C.: A one stage factor VIII (antihaemophilic globulin) assay and its use on venous and capillary plasma. Thromb. Diath. haemorrh.7, 215 (1962).
Hedner U. & Nilsson I. M.: Antithrombin III in a clinical material. Thromb. Res.3, 631 (1973).
Herman E. C., Jr. & Conley C. L.: Hereditary persistence of fetal hemoglobin. Amer. J. Med.29, 9 (1960).
McKay D. E.: Disseminated Intravascular Coagulation. An Intermediary Mechanism of Disease. Hoeber Medical Division, Harper and Row publ., New York 1965.
Mahmood A., MacIntosh D. M. & Sharper A. G.: Fibrinolytic activity in the clinical crises of sickle cell anaemia. Brit. med. J.3, 653 (1967).
Mannucci P. M., Lobina G. F., Caocci L. & Dioguardi N.: Effect on blood coagulation of massive intravascular haemolysis. Blood33, 207 (1969).
Merskey C., Kleiner G. J. & Johnson A. J.: Quantitative estimation of split products of fibrinogen in human serum; relation to diagnosis and treatment. Blood28, 1 (1966).
Odegard O. R., Abildgaard U. & Fegerhol M. K.: Antithrombin III related to the degree of hypercoagulation. IVth Intern. Congress on Thrombosis and Haemostasis (Abstract 190), Vienna 1973.
Penick G. O., Dejanov I. I., Roberts H. R. & Webster W. P.: Elevation of factor VIII in hypercoagulable states. Thromb. Diath. haemorrh (Suppl.)20, 39 (1966).
Rabiner S. F. & Friedman L. H.: The role of intravascular haemolysis and the reticuloendothelial system in the production of a hypercuagulable state. Brit. J. Haemat.14, 105 (1968).
Ratnoff O. D. & Menzie C.: A new method for the determination of fibrinogen in small samples of plasma. J. Lab. clin. Med.37, 316 (1951).
Sonnabend D., Cooper D., Fiddes P. & Penny R.: Fibrin degradation products in thromboembolic disease. Pathology4, 47 (1971).
von Kaulla K. N.: Chemistry of Thrombolysis: Human Fibrinolytic Enzymes. Thomas, Springfield Ill. 1963.
Williams W. I.: Disorders of hemostasis-thrombosis. In: Hematology, p. 1257. McGraw-Hill Book Co., New York 1972.
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Stathakis, N.E., Papayannis, A.G., Papayotas, H. et al. Hypercoagulability and hypofibrinolysis in sickle-cell disease. Blut 31, 355–360 (1975). https://doi.org/10.1007/BF01634001
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DOI: https://doi.org/10.1007/BF01634001