Department of Internal Medicine and Thrombosis Expert Center, Maastricht University Medical Center, Maastricht, The Netherlands.
CARIM School for Cardiovascular Diseases, Maastricht, The Netherlands.
Hamostaseologie. 2021 Apr;41(2):120-126. doi: 10.1055/a-1393-8302. Epub 2021 Apr 15.
Disseminated intravascular coagulation (DIC) is characterized by the intravascular activation of coagulation with loss of localization arising from different causes, and is diagnosed using scoring systems which rely upon the presence of an underlying disorder compatible with DIC alongside hemostatic derangements such as low platelet count, prolonged prothrombin time, and elevated fibrinogen degradation products. DIC is common in patients with acute leukemia, with prevalence ranging from 17 to 100% in acute promyelocytic leukemia (APL) and 8.5 to 25% in acute lymphoblastic leukemia (ALL) and non-APL acute myeloid leukemia (AML). The pathophysiology is complex and varies between the leukemia subtypes, and is not fully reflected by the laboratory markers currently used to classify DIC. Similarly, the clinical consequence of DIC in acute leukemia also varies across the types of leukemia. DIC is primarily associated with bleeding in APL, while thrombosis is the dominant phenotype in ALL and non-APL AML. The cornerstone of managing DIC is the treatment of the underlying disease, as exemplified by the important role of early administration of all-trans retinoic acid in APL. Other aspects of management focus on supportive care aimed at minimizing the risk of bleeding, via transfusion of blood products. The use of blood products is more liberal in APL, due to the hemorrhagic phenotype and unacceptably high rates of early hemorrhagic death. This review will focus on the pathophysiology, risk factors, clinical implications, and the management of DIC in patients across the spectrum of acute leukemias.
弥散性血管内凝血 (DIC) 的特征是由于不同原因导致的血管内凝血激活,失去定位,通过评分系统进行诊断,该系统依赖于与 DIC 相符的潜在疾病的存在以及止血紊乱,如血小板计数低、凝血酶原时间延长和纤维蛋白原降解产物升高。DIC 在急性白血病患者中很常见,在急性早幼粒细胞白血病 (APL) 中患病率为 17%至 100%,在急性淋巴细胞白血病 (ALL) 和非 APL 急性髓细胞白血病 (AML) 中患病率为 8.5%至 25%。其病理生理学复杂,在白血病亚型之间存在差异,并且目前用于分类 DIC 的实验室标志物并不能完全反映。同样,DIC 在急性白血病中的临床后果也因白血病类型而异。DIC 主要与 APL 的出血有关,而血栓形成是 ALL 和非 APL AML 的主要表型。管理 DIC 的基石是治疗基础疾病,如在 APL 中早期给予全反式维甲酸的重要作用。管理的其他方面侧重于支持性护理,旨在通过输血制品来最大程度地降低出血风险。由于出血表型和早期出血死亡的风险极高,因此在 APL 中更倾向于使用血液制品。本文将重点介绍 DIC 在各种急性白血病患者中的病理生理学、危险因素、临床意义和管理。