CAJ | 학술논문

目的：分析5例婴儿Kasabach-Merritt综合征（KMS）临床数据及转归。方法1993年6月-2012年2月期间我科收治的5例KMS患儿（男4例，女1例），回顾性收集上述病例的一般资料、血常规及出凝血检查、影像学和病理学检查结果并加以分析。结果5例患儿中3例为皮下型巨大血管瘤，2例为内脏型巨大血管瘤，除1例首诊时确诊为KMS外，其余4例首诊时均存在误诊。血液检查结果提示所有患儿均存在不同程度消耗性凝血功能障碍。所有患者均给予肾上腺糖皮质激素治疗及对症支持治疗，对激素敏感者3例，激素敏感率为60％，手术治疗1例。经治疗存活4例，死亡1例，死亡原因为严重DIC伴多脏器功能衰竭。结论应提高对KMS的认识以减少误诊发生率，对婴儿难治性血小板减少伴凝血功能异常者需注意除外KMS ，不同患儿对药物的治疗反应存在个体化差异，故应针对不同情况采用个体化综合治疗方法，以提高临床诊疗效果。
목적：분석5례영인Kasabach-Merritt종합정（KMS）림상수거급전귀。방법1993년6월-2012년2월기간아과수치적5례KMS환인（남4례，녀1례），회고성수집상술병례적일반자료、혈상규급출응혈검사、영상학화병이학검사결과병가이분석。결과5례환인중3례위피하형거대혈관류，2례위내장형거대혈관류，제1례수진시학진위KMS외，기여4례수진시균존재오진。혈액검사결과제시소유환인균존재불동정도소모성응혈공능장애。소유환자균급여신상선당피질격소치료급대증지지치료，대격소민감자3례，격소민감솔위60％，수술치료1례。경치료존활4례，사망1례，사망원인위엄중DIC반다장기공능쇠갈。결론응제고대KMS적인식이감소오진발생솔，대영인난치성혈소판감소반응혈공능이상자수주의제외KMS ，불동환인대약물적치료반응존재개체화차이，고응침대불동정황채용개체화종합치료방법，이제고림상진료효과。
Objective ToanalyzetheclinicaldataandprognosisonfivecaseofinfantKasabach-Merrittsyndrome(KMS).Methods Thegeneralinformation,andhaematological,pathological,and imaging results of five patients with Kasabach-Merritt syndrome between June 1 993 and February 201 2 were collectedforretrospectiveanalysis.Results Threepatientswerethegianthemangiomaofsubcutaneous type,two patients were the giant hemangioma visceral.In addition to one cases diagnosed as KMS,there were four cases of misdiagnosis of the rest of the first diagnosis.Haematological test showed that all patients had disseminated intravascular coagulation (DIC ).All patients were treated with glucocorticosteroid , symptomatic and supportive treatment.Three cases were sensitive to the glucocorticosteroid,hormone sensitve rate is 60%,one case received tumor excision.Four patients survived after treatment,one patient diedfromseriousDICandsubsequentmultipleorganfailure.Conclusions Toimprovecomprehensionof Kasabach-Merritt Syndrome and reduce the rate of misdiagnosis.KMS should be considered in patients presenting with refractory idiopathic thrombocytopenic and coagulant function abnormality.The existence of individual differences in treatment of drug reactions of different patients.So we should be aimed at different situation with individualized comprehensive treatment method to Improve clinical effect in patients with KMS.