中华皮肤科杂志 ›› 2014, Vol. 47 ›› Issue (11): 806-808.

• 研究报道 • 上一篇    下一篇

成人Kasabach-Merritt综合征一例临床及病理观察

吴晁1,鲁常青2,3,康定华4,孙军5   

  1. 1. 常州市第一人民医院病理科
    2.
    3. 常州市第一人民医院
    4. 江苏省常州市第一人民医院皮肤科
    5. 常州市第一人民医院影像科
  • 收稿日期:2014-02-10 修回日期:2014-03-11 出版日期:2014-11-15 发布日期:2014-11-01
  • 通讯作者: 吴晁 E-mail:cz2159@163.com

Clinicopathological analysis of a case of adult Kasabach-Merritt syndrome

  • Received:2014-02-10 Revised:2014-03-11 Online:2014-11-15 Published:2014-11-01

摘要: 患者女,52岁,不明原因血小板减少29年、右面颊部紫红色斑块9年。给予糖皮质激素等治疗后病情稍有好转,但血小板从未上升至正常,糖皮质激素减量后长期服中药调理。近来患者面颊部斑块肿胀疼痛,血小板进行性下降。血常规检查白细胞3.0 × 109/L,血红蛋白85 g/L,血小板2 × 109/L。CT检查示肝内多发血管瘤,其中肝左外叶1个14 cm × 5.7 cm。内科治疗后手术切除肝左外叶巨大血管瘤。术后血小板上升至120 × 109/L,面颊部肿胀消失。术后病理诊断:肝脏巨大海绵状血管瘤,血管腔内广泛血栓形成伴机化。综合分析,本例诊断:成人Kasabach-Merritt综合征。

关键词: Kasabach-Merritt综合征, 病例报告[文献类型]

Abstract: Wu Chao*, Lu Changqing, Kang Dinghua, Sun Jun. *Department of Pathology, First People′s Hospital of Changzhou, Third Affiliated Hospital of Suzhou University, Changzhou 213003, China 【Abstract】 A 52-year-old female presented with unexplained thrombocytopenia for 29 years and a prunosus plaque on the right cheek for 9 years. She had ever been treated with glucocorticoids, which resulted in a slight improvement of the condition, but the platelet count remained below the normal range. During glucocorticoid tapering, she had administrated traditional Chinese medicines for a long peroid. Recently, the plaque on the cheek became swollen and painful with a progressive decrease in platelet count. Routine blood test showed that the count of white blood cells and platelets was 3.0 × 109/L and 2 × 109/L respectively, and the concentration of hemoglobin was 85 g/L. Computed tomography (CT) scan revealed multiple liver hemangiomas, one of which in the left liver lobe measured 14 cm × 5.7 cm in size. After medical treatment, the giant hemangioma in the left lobe was resected. Thereafter, the platelet count increased to 120 × 109/L with the subsidence of swelling of the right cheek. Postoperative pathological examination confirmed a diagnosis of giant cavernous hemangioma of the liver with extensive intravascular thrombosis and thrombus organization. Based on these findings, the case was diagnosed as adult Kasabach-Merritt syndrome.

Key words: Kasabach-Merritt syndrome, Case reports [Publication type]