HEREDITARY HEMORRHAGIC TELANGIECTASIA WITH MALIGNANT LYMPHOMA An Autopsy Case
HEREDITARY HEMORRHAGIC TELANGIECTASIA WITH MALIGNANT LYMPHOMA An Autopsy Case
复制标题
遗传性出血性毛细血管扩张症伴恶性淋巴瘤尸检案例
DOI：
10.1111/j.1440-1827.1988.tb02310.x
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发表时间：
1988
影响因子：
2.2
通讯作者：
K. Ichihara
中科院分区：
文献类型：
--
作者：
Shinsuke Kato;K. Ichihara
一名60岁的日本女性在尸检时被诊断为患有与全身性血管瘤相关的遗传性出血性毛细血管扩张症（HHT）。在生育期内，经前鼻出血频繁。60岁时，患者死于恶性淋巴瘤。尸检时，在面部、四肢和躯干上发现多个毛细血管扩张点。主动脉旁淋巴结肿大，不规则聚集，组织学诊断为弥漫性大细胞型恶性淋巴瘤。粘膜下毛细血管扩张性病变见于从口腔到直肠的胃肠系统。海绵状血管瘤存在于各种内脏器官，包括肝脏、脾脏、小肠和大肠、直肠、阑尾、子宫、空肠和结肠肠系膜。左肺有动静脉瘘。对她的家庭系谱的检查表明，病人有一个常染色体显性遗传特征。在这个病人中观察到的全身内脏血管瘤的发病机制被认为是类似于错构瘤。
A 60‐year‐old Japanese woman was diagnosed at autopsy as having had hereditary hemorrhagic telangiectasia (HHT) associated with systemic hemangiomas. In her repoduction period, premenstrual epistaxis frequently occurred. At the age of 60, the patient died of malignant lymphoma. At autopsy, multiple telangiectatic spots were noted on the face, limbs and trunk. The paraaortic lymph nodes, which were enlarged and irregularly conglomerated, were histologically diagnosed as malignant lymphoma of the diffuse large cell type. Submucosal telangiectatic lesions were found in the gastrointestinal system from the oral cavity to the rectum. Cavernous hemangiomas were present in various visceral organs including the liver, spleen, small and large intestines, rectum, appendix, uterus, and jejunal and colonic mesenteries. There was an arteriovenous fistula in the left lung. Examination of her family pedigree showed that the patient had an autosomal dominant trait of inheritance. The pathogenesis of the systemic visceral hemangiomas observed in this patient was considered to be similar to that of hamartoma.