Choeyprasert Worawut, Natesirinilkul Rungrote, Charoenkwan Pimlak
Department of Pediatrics, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand.
Case Rep Hematol. 2014;2014:364693. doi: 10.1155/2014/364693. Epub 2014 May 22.
Kasabach-Merritt phenomenon (KMP) is relatively rare in childhood and adolescents with high mortality rate because of its hemorrhagic complications and unresponsiveness to treatments such as corticosteroids, vincristine, intravascular embolization, and/or surgery. Propranolol, a β -adrenergic receptor blocker, has a promising efficacy against vascular tumors such as infantile hemangiomas. But limited and variable data has been reported regarding the role of propranolol in treatment of KMP. We herein reported the successful treatment of mild pediatric KMP with propranolol monotherapy in a case of a five-week-old child with kaposiform hemangioendothelioma with successful treatment of both clinical and hematologic responses. After eight months of follow-up, patient still had stable cutaneous lesion while receiving propranolol monotherapy. Regular hematologic monitoring was done in order to detect any late relapse of the disease. Six months after discontinuation of propranolol, patient has still remained free of hematologic relapse, and primary cutaneous lesion has become a pale pink, 1 cm sized skin lesion.
卡萨巴赫-梅里特现象(KMP)在儿童和青少年中相对罕见,由于其出血性并发症以及对皮质类固醇、长春新碱、血管内栓塞和/或手术等治疗无反应,死亡率很高。普萘洛尔是一种β肾上腺素能受体阻滞剂,对婴儿血管瘤等血管肿瘤具有良好的疗效。但关于普萘洛尔在KMP治疗中的作用,报道的数据有限且存在差异。我们在此报告了一例5周大患有卡波西样血管内皮瘤的儿童,采用普萘洛尔单一疗法成功治疗轻度小儿KMP,临床和血液学反应均取得成功。经过8个月的随访,患者在接受普萘洛尔单一疗法时皮肤病变仍稳定。进行定期血液学监测以检测疾病的任何晚期复发。停用普萘洛尔6个月后,患者仍未出现血液学复发,原发性皮肤病变已变为淡粉色、1厘米大小的皮肤病变。