Department of Pediatrics, Division of Hematology/Oncology, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA.
Arkansas Children's Hospital, Little Rock, Arkansas, USA.
Pediatr Blood Cancer. 2022 Aug;69 Suppl 3:e29353. doi: 10.1002/pbc.29353.
Some vascular anomalies can present with challenging hematologic aberrations. Kaposiform hemangioendothelioma (KHE) may be complicated with Kasabach-Merritt phenomenon (KMP) and stagnant blood flow in slow-flow malformations can promote activation and consumption of coagulation factors, which results in bleeding and clotting known as localized intravascular coagulopathy (LIC). These patients can experience significant morbidity secondary to pain due to thrombosis and are at higher risk of hematologic complications during surgical procedures. No standard of care has been established to prevent or manage these complications. This review focuses on the management of coagulopathy in children and adults with vascular anomalies.
一些血管异常可能伴有具有挑战性的血液学异常。卡波西样血管内皮细胞瘤(KHE)可能并发卡-梅现象(KMP),并且慢血流畸形中的血流停滞会促进凝血因子的激活和消耗,导致出血和凝块,即局部血管内凝血(LIC)。这些患者由于血栓形成而疼痛,可能会出现严重的发病率,并在手术过程中更易发生血液学并发症。目前尚未建立预防或处理这些并发症的标准。本综述重点介绍血管异常儿童和成人的凝血异常管理。