Szlachetka D M
Department of Pediatrics, Tufts University School of Medicine, USA.
Neonatal Netw. 1998 Feb;17(1):7-15.
Hemangiomas are common newborn vascular tumors occurring in up to 2.5 percent of newborns. Most are benign and 70 to 80 percent regress by age seven. Some hemangiomas are life threatening--1 in 300 is associated with coagulopathy. Kasabach-Merritt syndrome (KMS), associated with hemangioma, disseminated intravascular coagulopathy, microangiopathic anemia, and thrombocytopenia, can have profound sequelae. Morbidity and mortality are influenced by the anatomic location and size of the hemangioma. Untreated KMS has a 10-37 percent mortality rate. Bleeding secondary to the consumptive coagulopathy is the primary cause of death in these patients. As of 1997, there have been approximately 205 cases of KMS reported in the literature. This case study discusses the pathophysiology, clinical manifestations, diagnostics, treatment modalities, differential diagnosis, and psychosocial considerations of KMS.
血管瘤是常见的新生儿血管肿瘤，发生率高达2.5%。大多数为良性，70%至80%在7岁前会自行消退。一些血管瘤会危及生命——每300例中有1例与凝血病有关。与血管瘤相关的卡萨巴赫-梅里特综合征（KMS），伴有弥散性血管内凝血、微血管病性贫血和血小板减少症，可产生严重后果。发病率和死亡率受血管瘤的解剖位置和大小影响。未经治疗的KMS死亡率为10%至37%。这些患者死亡的主要原因是消耗性凝血病继发的出血。截至1997年，文献中报道的KMS病例约有205例。本病例研究讨论了KMS的病理生理学、临床表现、诊断、治疗方式、鉴别诊断以及社会心理因素。
