新生儿Kasabach-Merritt综合征5例报道并文献复习

Kasabach-Merritt syndrome in neonates: report of 5 cases and review of the literatures.

目的提高对Kasabach-M erritt综合征的认识。方法结合5例Kasabach-M erritt综合征患儿的临床资料和文献复习,详细分析该病的病因、临床表现、诊断、治疗及预后等。结果该病病因不清。临床表现多样化;病理检查主要为毛细血管瘤、Kaposiform血管内皮瘤、蔓状血管瘤。治疗目前无单一治疗措施,主要是去除病因,纠正D IC和血小板减少,包括激素、肝素的应用和支持疗法等。血管瘤的治疗分为药物治疗和非药物治疗,须根据具体情况而定。结论婴儿血管瘤尤其是巨大血管瘤时应警惕Kasabach-M erritt综合征的发生。治疗的关键是去除病因。如不能及时控制D IC,往往使得治疗棘手。

Objective： To described the clinical features of Kasabach -Merritt syndrome. Methods： With report of 5 cases and review of the related literatures, the etiology, clinical manifestations, diagnosis, differential diagnosis, management and prognosis of Kasabach - Merritt syndrome were described. Results ： The etiology of this disease remains unknow. The cases pulished in the world literature illustrate the diversity of clinical presentation and unpredictable response to therapy. The histological type most frequently reported were that of kapesiform haemangioendothelioma, tufted angiomas and infantile capillary haemangioma. There are many therapies for KMS, but the mainstays of those are the resolution of tumor and manage the coagulopathy and thrombocytopenia, which include administration of corticosteroids and heparin, supportive therapy and so on. Deciding which therapies to use, pharmacological or non - pharmacological treatment, depends on the clinical setting of patients initially and the response subsequently. Conlusion： KMS should always be considered in patients presenting with a giant haemangioma and thrombocytopenia. The premise regarding treatment of KMS is that resolution of the lesion will lead to a correction of the consumption coagulopathy, although the therapeutic dilemmas are similar to managing disseminated intravascular coagulation （DIC）.