伴Kasabach-Merritt现象卡波西形血管内皮瘤和丛状血管瘤的临床及病理特征 |
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引用本文: | 陈辉,林晓曦,林梅绥,李伟,金云波,马刚,胡晓洁,周兆平.伴Kasabach-Merritt现象卡波西形血管内皮瘤和丛状血管瘤的临床及病理特征[J].中华皮肤科杂志,2009,42(8):674-676. |
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作者姓名: | 陈辉 林晓曦 林梅绥 李伟 金云波 马刚 胡晓洁 周兆平 |
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作者单位: | 200011上海交通大学医学院附属第九人民医院速整复外科;200011上海交通大学医学院附属第九人民医院病理科; |
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摘 要: | Objective To investigate the clinical and pathological features of Kasabach-Merritt phenomenon(KMP)associated with Kaposiform hemangioendothelioma(KHE)and tufted angioma(TA).Methods Clinical presentations and pathological features of seven patients with KHE or TA complicated by KMP were analyzed retrospectively and a literature review was made.Results Five patients were diagnosed with KHE and two with TA,and all of them exhibited a decrease in platelet count(less than 100×109/L).In all patients,lesions were present or developed on the trunk or limbs at birth or wimin one month after birth.The lesions showed a rapid growth in two patients,slow expansion in two patients,kept stable or regressed gradually in three patients.Lesion appearance varied in KHE and TA,and characteristic manifestation was dark-erythematous or prunosus tumors or patches involving the skin or subcutis.Histopathologically.both KHE and TA were composed of numerous fusiform endothellal cell clumps arranged in a lobular fashion.The nodules of KHE had an indistinct boundary usually with erythrocyte stasis or hemosiderin deposition,and TA was characterized by rounded,well-defined nodules distributed in a crater-like fashion within the dermis.A satisfactory outcome was achieved in two patients treated with oral dehydrocortisone and one patient with surgical resection plus skin grafting;the other four patients remained untreated and no progress was observed during follow-up.Conclusions KMP is pathologically associated with KHE or TA.KHE is an intermediate tumor with local aggressiveness while TA is a benign tumor.Both of them can regress spontaneously and should be difierentiated from other types of vascular tumors.
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关 键 词: | Kasabach-Merritt 现象 血管内皮瘤 血管瘤 |
Clinical and pathological features of Kasabach-Merritt phenomenon associated with Kaposiform hemangioendothelioma and tufted angioma |
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Keywords: | Kasabach-Merritt phenomenonHemangioendotheliomaHemangioma |
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