Karpatkin M
New York University School of Medicine, NY, USA.
Pediatr Clin North Am. 1971 Feb;18(1):23-38. doi: 10.1016/s0031-3955(16)32520-2.
Disseminated intravascular coagulation (defibrination syndrome, consumption coagulopathy) is a syndrome which may complicate a number of pathologic states. If clotting factors and platelets are "consumed" more rapidly than the patient can produce them, they may fall to levels which are not adequate for hemostasis. Thus, thrombosis with resulting necrosis and hemorrhage may occur simultaneously in the same patient. Local fibrinolysis (secondary fibrinolysis) may remove or partially remove intravascular thrombi, thus protecting against local tissue necrosis. Diagnosis of the fulminating syndrome is usually simple and depends upon a few relatively easy tests. If the syndrome is low grade, diagnosis is very difficult and may be impossible without sophisticated techniques. Differential diagnosis from coagulation defects secondary to liver disease is extremely difficult. Treatment in the fulminating case before intravascular thrombi can cause irreversible tissue damage is urgent. Treatment depends upon therapy of the underlying disease plus adequate heparinization. It may be necessary to administer platelets and blood fractions after heparinization. Corticosteroids are not contraindicated, particularly when the patient is heparinized. Epsilon-amino-caproic acid (EACA, Amicar) is contraindicated. This drug inhibits local fibrinolysis, which is protective to the patient.
弥散性血管内凝血（去纤维蛋白综合征、消耗性凝血病）是一种可能并发于多种病理状态的综合征。如果凝血因子和血小板的“消耗”速度超过患者的生成速度，它们可能降至不足以实现止血的水平。因此，血栓形成以及随之而来的坏死和出血可能在同一患者体内同时发生。局部纤维蛋白溶解（继发性纤维蛋白溶解）可清除或部分清除血管内血栓，从而防止局部组织坏死。暴发性综合征的诊断通常很简单，取决于一些相对简单的检查。如果综合征为轻度，诊断非常困难，若无复杂技术可能无法诊断。与肝病继发的凝血缺陷进行鉴别诊断极其困难。在血管内血栓导致不可逆组织损伤之前，对暴发性病例进行治疗十分紧迫。治疗取决于对基础疾病的治疗以及充分的肝素化。肝素化后可能需要输注血小板和血液成分。皮质类固醇并非禁忌，尤其是在患者接受肝素化治疗时。6-氨基己酸（EACA，止血芳酸）是禁忌的。这种药物会抑制对患者有保护作用的局部纤维蛋白溶解。
