Keskindemirci Gonca, Tuğcu Deniz, Aydoğan Gönül, Akçay Arzu, Aktay Ayaz Nuray, Er Ali, Yekeler Ensar, Bilgiç Bilge
Department of Pediatric Hematology-Oncology, İstanbul Kanuni Sultan Süleyman Educational and Research Hospital, 34303 Istanbul, Turkey.
Department of Pediatric Hematology-Oncology, Faculty of Medicine, Acıbadem University, 34742 Istanbul, Turkey.
Case Rep Pediatr. 2015;2015:537530. doi: 10.1155/2015/537530. Epub 2015 Sep 8.
Kasabach-Merritt phenomenon (KMP) is characterized by vascular tumour and consumptive coagulopathy with life-threatening thrombocytopenia, prolonged prothrombin time and partial thromboplastin time, hypofibrinogenemia, and the presence of high fibrin split products. We report a case of 3-year-old boy with local aggressive vascular lesions associated with KMP. Magnetic resonance imaging revealed an extensive lesion at paravertebral and retroperitoneal regions that was infiltrating vertebrae. Although we did not get any response to steroid or propranolol treatment, partial response was observed radiologically with interferon-alpha treatment. Unfortunately, the patient died because of the uncontrolled consumptive coagulopathy that led to intracranial hemorrhage which was caused by huge knee hematoma after minor trauma.
卡萨巴赫-梅里特现象(KMP)的特征是血管肿瘤和消耗性凝血病,伴有危及生命的血小板减少、凝血酶原时间和部分凝血活酶时间延长、纤维蛋白原血症降低以及高纤维蛋白裂解产物的存在。我们报告一例3岁男孩,患有与KMP相关的局部侵袭性血管病变。磁共振成像显示椎旁和腹膜后区域有广泛病变,该病变正在浸润椎体。尽管我们对类固醇或普萘洛尔治疗没有任何反应,但在接受α干扰素治疗后,影像学上观察到部分反应。不幸的是,患者因消耗性凝血病失控导致颅内出血而死亡,颅内出血是由轻微创伤后巨大的膝部血肿引起的。