Zhang Deng-Yong, Lu Zheng, Ma Xiang, Wang Qiu-Yue, Sun Wang-Liang, Wu Wei, Cui Pei-Yuan
From the Department of Hepatobiliary Surgery, First Affiliated Hospital of Bengbu Medical College (D-YZ, ZL, XM, W-LS,WW, P-YC); Department of Bengbu Medical College, Bengbu, Anhui, China (Q-YW).
Medicine (Baltimore). 2015 Jul;94(27):e1126. doi: 10.1097/MD.0000000000001126.
Hemolymphangioma is a rare disease with malformation of both lymphatic and vascular vessels. Few cases of hemolymphangioma occurring in the rectum, small intestine, pancreas, esophagus, and other organs have been reported. Nevertheless, multiple hemolymphangioma of the visceral organs are extremely rare. We report a 25-year-old female with a significantly enlarged spleen full of multiple-rounded lesions. Curiously, the splenic flexure and even retroperitoneum had many lesions. The patient recovered well after splenectomy and the pathologic diagnosis of spleen was hemolymphangioma with abnormal lymphatic and blood vessels with polycystic spaces.Usually, it is hard to cure this disease. We should take much more consideration into the diagnosis, treatment, and even pathogenesis, even though it is a benign lesion.
血淋巴管瘤是一种淋巴管和血管均发生畸形的罕见疾病。直肠、小肠、胰腺、食管等器官发生血淋巴管瘤的病例鲜有报道。然而,内脏器官多发性血淋巴管瘤极为罕见。我们报告一例25岁女性患者,其脾脏显著增大,布满多个圆形病灶。奇怪的是,脾曲甚至腹膜后也有许多病灶。患者脾切除术后恢复良好,脾脏病理诊断为血淋巴管瘤,伴有异常淋巴管和血管以及多囊性间隙。通常,这种疾病很难治愈。尽管它是良性病变,但我们在诊断、治疗甚至发病机制方面都应给予更多考虑。