Musumeci S, Leonardi S, Di Dio R, Fischer A, Di Costa G
Acta Haematol. 1987;77(1):30-3. doi: 10.1159/000205945.
Seventy-four patients with beta-thalassemia major were studied to test the hypothesis that a deficiency of protein C (PC) and antithrombin III (AT III), both antithrombotic proteins, could contribute to the pathogenesis of CNS thromboembolic lesions. In 70 patients, PC levels were found to be significantly lower than normal, whereas AT III activity was found to be lower only in 41 patients. The lowest values of PC and AT III were found in older splenectomized patients, a low PC value only was found in chronic hepatitis patients. Prothrombin time and fibrinogen were found to be particularly abnormal in patients with chronic hepatitis and without spleen. A relatively poor correlation was observed between PC and AT III (p less than 0.02). PC correlated with age (p less than 0.001), transfusional iron (p less than 0.001) and ferritin (p less than 0.001). It also correlated with serum albumin (p less than 0.001), prothrombin time (p less than 0.001) and fibrinogen (p less than 0.02) and with serum transaminases (GPT) (p less than 0.001). The same indexes correlated less significantly with AT III activity. Nevertheless, only 2 of our patients had CNS thromboembolic complications. It is probable that low clotting factors, hyperfibrinolysis and thrombocytopenia (which are common in chronic liver disease) could have the opposite effect on hemostasis from that of low levels of anticoagulant proteins such as PC and AT III.
对74例重型β地中海贫血患者进行了研究，以检验以下假设：抗血栓蛋白蛋白C（PC）和抗凝血酶III（AT III）的缺乏可能会导致中枢神经系统血栓栓塞性病变的发病机制。在70例患者中，发现PC水平显著低于正常水平，而仅在41例患者中发现AT III活性降低。PC和AT III的最低值出现在年龄较大的脾切除患者中，仅在慢性肝炎患者中发现PC值较低。在慢性肝炎且无脾脏的患者中，凝血酶原时间和纤维蛋白原尤其异常。观察到PC和AT III之间的相关性相对较差（p<0.02）。PC与年龄（p<0.001）、输血铁（p<0.001）和铁蛋白（p<0.001）相关。它还与血清白蛋白（p<0.001）、凝血酶原时间（p<0.001）、纤维蛋白原（p<0.02）以及血清转氨酶（GPT）（p<0.001）相关。相同的指标与AT III活性的相关性较低。然而，我们的患者中只有2例出现中枢神经系统血栓栓塞并发症。很可能低凝血因子、高纤维蛋白溶解和血小板减少（这些在慢性肝病中很常见）对止血的影响与低水平抗凝蛋白如PC和AT III的影响相反。
