成人多发Kasabach—Merritt综合征的诊治体会.DOCVIP

成人多发Kasabach—Merritt综合征? 摘要目的探讨Kasabach—Merritt综合征临床特点、诊断及治疗方法。方法 1例成人 KMS患者的，。结临床表现以全身多处血管瘤、出血倾向伴继发性溶亢进为主，影像学对这种疾病的诊断价值较大，治疗方案以内科治疗和手术治疗为主。我们采取间断输注血浆、血小板及对症治疗取得较好的效果。结论 是一种罕见疾病，临床表现不典型，极易误诊和漏诊，并且治疗效果有限、预后差，需进一步提高该疾病的诊断和治疗水平。 关键词 Kasabach—Merritt综合征 诊断 成人dult multiple kasabach–merritt syndrome Objective: To report a case of adult Kasabach–Merritt syndrome and explore the clinical characteristics, diagnosis and treatment strategies of this rare disease. Methods: A case of Kasabach–Merritt syndrome was presen-ted ;the experience of diagnose and treatment was discussed. Results: The main clinical characteristics of?KMS include the decrease in platelet count, bleeding tendency?with secondary?hyperfibrinolysis?.The radiological findings are valueable for KMS. The current?therapy?for this disease mainly relies on medication and surgical treatment. For this case, we performed?intermittent?infusion?of plasma,?platelets along with?other supportive treatments and the patient showed good response. Conclusions: KMS is very rare. The clinical?manifestation of this disease is not always typical,?thus frequently?being misdiagnosed.?The efficacy of relatively limited treatment options is not ideal,?and the prognosis of patients is poor.?Further research on this rare disease is in need. Key words kasabach–merritt syndrome;?diagnosis;Adult ? ? Kasabach—Merritt综合征，文献报道以婴幼儿居多，成人罕见。目前国内文献报道成人KMS仅9例[1-8]。国外报道婴幼儿病例多见，成人发病罕见。 ?临床资料? ? ?? ? ?患者，女，43岁。因“鼻腔间断出血两年，头晕乏力一周”入院。于2012年8月份开始无明显诱因出现鼻腔间断出血，每次约2ml-5ml可自行停止，无其他部位出血，：白细胞3.×109/L，红细胞3.3×1012/L血红蛋白 98. G/L，血小板计数 3×109/L，中性粒细胞绝对值1.9×109/L未行特殊治疗。门诊以血小板减少原因待查入院。既往幼年时因背部软组织包块行切除术，术后病理活检为良性包块（具体不详）；否认其他病史。查体：下唇正中有一血泡，约0.5×0.5cm大小右侧小腿胫前有一小瘀斑约3×4cm其余皮肤未见出血症，巩膜轻度黄染，浅表淋巴结未触及肿大，右侧锁骨、背部、右侧肘部及鼻唇沟分别可触及多个软组织包块，质地软，无压痛，边界清，背部最大包块约10×12cm，双肺呼吸音清，73bpm，律齐，腹部平软，肝肋下4.4cm表面光滑，质中，无压痛，脾肋下1.0cm，质韧、无压痛。：白细胞3.×109/L，红细胞3.39×1012/L血红蛋白 98.8 G/L血小板计数 34×109/L中性粒细胞绝对值1.96×109/L。乙肝表面抗原阳性。肝功能：总胆红素53.1 μmol/L直接胆红素12.5 μmol/L间接胆红素40.6μmol/L肾功能正常。凝血酶原时间（PT）13.3s（9-