Crary Shelley E
Pediatric Hematology-Oncology, University of Arkansas for Medical Sciences, Little Rock, AR.
Hematology Am Soc Hematol Educ Program. 2024 Dec 6;2024(1):718-723. doi: 10.1182/hematology.2024000597.
The role of the hematologist in the management of vascular anomalies is evolving. Several vascular tumors and malformations are associated with complex coagulation derangements. Kaposiform hemangioendothelioma or tufted angiomas may present with a consumptive coagulopathy known as the Kasabach-Merritt phenomenon (KMP). The management of KMP is essential to reduce morbidity and mortality from this condition. Slow-flow vascular malformations (SFVM) are also frequently complicated by a coagulopathy requiring anticoagulation, especially during and after surgical procedures, and some of these conditions pose a high risk of venous thromboembolism. Pain in SFVM is also frequently responsive to anticoagulation as well. It is essential for a hematologist with expertise in vascular anomalies to assist in the management of these complex conditions as part of a multidisciplinary team to reduce morbidity and mortality. Through case-based discussions, we attempt to highlight the critical role of the hematologist in managing these anomalies.
血液科医生在血管异常管理中的作用正在不断演变。几种血管肿瘤和畸形与复杂的凝血紊乱有关。卡波西样血管内皮瘤或簇状血管瘤可能会出现一种称为卡萨巴赫-梅里特现象（KMP）的消耗性凝血病。KMP的管理对于降低这种疾病的发病率和死亡率至关重要。缓慢血流性血管畸形（SFVM）也经常并发需要抗凝治疗的凝血病，尤其是在手术过程中和手术后，其中一些情况还存在静脉血栓栓塞的高风险。SFVM引起的疼痛通常对抗凝治疗也有反应。血管异常方面的血液科专家作为多学科团队的一员协助管理这些复杂情况以降低发病率和死亡率至关重要。通过基于病例的讨论，我们试图强调血液科医生在管理这些异常中的关键作用。
