Farid Mohamad, Ahn Linda, Brohl Andrew, Cioffi Angela, Maki Robert G
Mount Sinai Medical Center, One Gustave L. Levy Place, P.O. Box 1128, New York, NY 10029-6574, USA.
Sarcoma. 2014;2014:617102. doi: 10.1155/2014/617102. Epub 2014 Feb 13.
Objectives. To report the prevalence of consumptive coagulopathy in angiosarcoma patients seen at a single center. Methods. We retrospectively reviewed case records of 42 patients diagnosed with angiosarcoma at Mount Sinai Hospital between 2000 and 2013. Results. Seven patients (17%) met clinical criteria for disseminated intravascular coagulation (DIC) in absence of concomitant clinical states known to cause coagulopathy or myelosuppression. In all patients who received systemic antineoplastic therapy with resultant disease response or stability, DIC resolved in tandem with clinical improvement. DIC recurred at time of disease progression in all cases. Two patients had bulky disease, defined as diameter of largest single or contiguous tumor mass measuring 5 cm or more. All patients demonstrated an aggressive clinical course with short duration of disease control and demise within 1 year. In contrast, evaluation over the same period of 17 epithelioid hemangioendothelioma patients serving as a clinical control group revealed no evidence of DIC. Conclusion. Angiosarcomas can be associated with a consumptive coagulopathy arising in tandem with disease activity. Vigilance for this complication will be needed in the course of often aggressive multimodality therapy. The potential utility of coagulopathy as a prognostic biomarker will need to be explored in future studies.
目的。报告在单一中心就诊的血管肉瘤患者中消耗性凝血病的患病率。方法。我们回顾性分析了2000年至2013年在西奈山医院确诊为血管肉瘤的42例患者的病例记录。结果。7例患者（17%）符合弥散性血管内凝血（DIC）的临床标准，且不存在已知可导致凝血病或骨髓抑制的伴随临床状态。在所有接受全身抗肿瘤治疗并获得疾病缓解或病情稳定的患者中，DIC随着临床症状的改善而缓解。在所有病例中，DIC在疾病进展时复发。2例患者有巨大肿块，定义为最大单个或相邻肿瘤肿块直径≥5 cm。所有患者临床病程均呈侵袭性，疾病控制时间短，1年内死亡。相比之下，同期对作为临床对照组的17例上皮样血管内皮瘤患者进行评估，未发现DIC证据。结论。血管肉瘤可伴有与疾病活动相关的消耗性凝血病。在通常积极的多模式治疗过程中，需要警惕这种并发症。凝血病作为一种预后生物标志物的潜在效用有待未来研究探索。
