發(fā)布時間：2018-07-25 10:02

【摘要】：目的討論卡梅現(xiàn)象(KMP)的臨床表現(xiàn)、診斷與治療。方法回顧分析2010年1月-2016年1月收治的13例卡梅現(xiàn)象患兒的臨床資料,并進(jìn)行文獻(xiàn)復(fù)習(xí)。結(jié)果 13例患兒中男10例、女3例,發(fā)病年齡均6個月。12例患兒有出血點(diǎn)、瘀斑、黃疸和皮膚包塊等皮膚表現(xiàn),1例表現(xiàn)為胸腔積液;病灶位置分布多樣;血小板嚴(yán)重減少,纖維蛋白原減少,D-二聚體升高;起病至確診中位時間為60天。采用手術(shù)及糖皮質(zhì)激素、心得安、干擾素、西羅莫司等藥物治療,10例緩解、3例死亡,其中6例給予西羅莫司治療者療效均滿意。結(jié)論卡梅現(xiàn)象以血管腫瘤合并嚴(yán)重血小板減少及消耗性凝血異常為特點(diǎn),發(fā)病年齡小,起病至確診時間較長;手術(shù)治療療效確切;西羅莫司或可成為卡梅現(xiàn)象的有效治療手段。
[Abstract]:Objective to discuss the clinical manifestations, diagnosis and treatment of kame phenomenon (KMP). Methods to review and analyze the clinical data of 13 cases of kame phenomenon in January January 2010 -2016, and review the literature. Results 13 children were 10 males and 3 women, and the age of the onset of.12 had bleeding spots, ecchymosis, jaundice and skin mass. At present, 1 cases were pleural effusion, the location of the lesion was varied, the platelets were severely reduced, the fibrinogen was reduced, the D- two polymer was raised, the onset to the median time of diagnosis was 60 days. The operation and glucocorticoid, propranolol, interferon, sirolimus, 10 cases of remission, and 3 cases of death were used, of which 6 cases were treated with sirolimus. Conclusion kame phenomenon is characterized by vascular tumor associated with severe thrombocytopenia and depleting coagulopathy. The onset age is small, the onset of the disease is longer, and the operative treatment is effective. Cicrolimus may be an effective treatment for the phenomenon of Kali.
【作者單位】： 上海交通大學(xué)醫(yī)學(xué)院附屬上海兒童醫(yī)學(xué)中心血液腫瘤科;
【分類號】：R732.2

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