Padua y Gabriel A, Peraza-Martínez L, Peralta-Sánchez J, Jaramillo Y, Arista-Nasr J
Servicio de Neumología, Centro Médico Nacional, Torreón, Coahuila.
Rev Invest Clin. 1994 Jul-Aug;46(4):301-5.
The clinicopathologic characteristics of two patients with Gorham's disease and bilateral recidivant pleural effusions are reported. The first case corresponded to a nine year-old girl with osteolysis in her right clavicle and ribs. The second case was a 55 year-old male with osteolytic lesions in ribs, cranium, pelvis and femurs; a thymic cyst was also documented. In both patients, biopsies were performed from ribs, which showed proliferating, benign, thin walled small vessels that extensively replaced the intertrabecular spaces. Thoracocentesis, instillation of talcum and radiation therapy were used in the former patient and no recidivant pleural effusions have developed after one year. The second patient was not treated with radiation therapy, persisted with pleural effusions and finally died two years after the diagnosis. Because Gorham's disease is rare, it was not immediately suspected in the cases described here. Its association with pleural effusion is even rarer and has been mentioned only occasionally in the literature. The utility of radiation therapy in Gorham's disease is commented.
报告了两名戈勒姆病患者伴有双侧复发性胸腔积液的临床病理特征。第一例为一名9岁女孩，右锁骨和肋骨出现骨质溶解。第二例是一名55岁男性，肋骨、颅骨、骨盆和股骨出现溶骨性病变；还记录到一个胸腺囊肿。两名患者均对肋骨进行了活检，结果显示有增生的、良性的、薄壁小血管广泛取代小梁间隙。对前者患者采用了胸腔穿刺、滑石粉灌注和放射治疗，一年后未出现复发性胸腔积液。第二例患者未接受放射治疗，胸腔积液持续存在，最终在诊断后两年死亡。由于戈勒姆病罕见，在此描述的病例中最初未被怀疑。其与胸腔积液的关联更为罕见，仅在文献中偶尔被提及。文中对放射治疗在戈勒姆病中的效用进行了评论。
