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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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