Kato S, Ichihara K
Second Department of Pathology, Tottori University School of Medicine, Yonago, Japan.
Acta Pathol Jpn. 1988 Mar;38(3):383-91. doi: 10.1111/j.1440-1827.1988.tb02310.x.
A 60-year-old Japanese woman was diagnosed at autopsy as having had hereditary hemorrhagic telangiectasia (HHT) associated with systemic hemangiomas. In her reproduction 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 harmartoma.
一名60岁的日本女性在尸检时被诊断患有遗传性出血性毛细血管扩张症（HHT）并伴有全身性血管瘤。在她的育龄期，经常出现经前鼻出血。60岁时，该患者死于恶性淋巴瘤。尸检时，在面部、四肢和躯干发现多个毛细血管扩张斑。主动脉旁淋巴结肿大且不规则聚集，组织学诊断为弥漫大细胞型恶性淋巴瘤。从口腔到直肠的胃肠道系统发现黏膜下毛细血管扩张性病变。包括肝脏、脾脏、小肠和大肠、直肠、阑尾、子宫以及空肠和结肠系膜在内的各种内脏器官均存在海绵状血管瘤。左肺有动静脉瘘。对其家族谱系的检查表明，该患者具有常染色体显性遗传特征。该患者全身性内脏血管瘤的发病机制被认为与错构瘤相似。
