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Anomalies of coagulation suggestive of a hypercoagulabe state have been extensively documented in sickle cell diseases (SS). They include decreased thrombin formation inhibitors: protein C (PC) and protein S (PS) and increased markers of thrombin formation such as prothrombin fragment 1.2 (F1.2). Their role in the pathogenesis of stroke is still controversial. Chronic transfusion prevents recurrence of stroke, or new stroke in patients identified at high risk by transcranial Doppler. If coagulation abnormalities leading to an hypercoagulable state plaid a major role in the pathogenesis of stroke in SCD, it would be expected that these would be corrected by a chronic transfusion regimen and would not be present in chronically transfused patients who do not have increased incidence of stroke, such as Thalassemics patients (Thal). We measured PC, PS (functional, free and total) and F1.2 in 106 SS, 16 SS patients on chronic transfusion (SSTx: mean Hb S = <20%) and 11 Thal. PC was decreased in all groups. PS was low in the SS patients and lowest in the SSTx. PS and F1.2 were correlated with total Hb and with WBC in the SS (PS free: p =0.0002, PS functional: p <0.0001, PS total: p =0.02, F1.2: p=0.004). None of these correlations persisted in the Tx SS or in the Thal. PC was not correlated with any hematological parameter. F1.2, although more often elevated in non-transfused SS was also elevated in the Thal. Since low PS and increased F1.2 are related to the degree of anemia and WBC, they reflect overall severity of SS rather then a prediposition to stroke. We conclude that low PC and PS and elevated F1.2 are not important factor in the pathogenesis of stroke in SS, since similar abnormalities are also found in the SSTx and in Thal who are not at risk of stroke. Table
Hurlet, A., Piomelli, S. & Martin, B. In Sickle Cell Disease Patients Coagulation Abnormalities Are Not A Major Predisposing Factor To Stroke † 768.
Pediatr Res43
(Suppl 4), 133 (1998). https://doi.org/10.1203/00006450-199804001-00789
