Geramizadeh Bita, Fattahi Mohammad Reza, Bagheri Lankarani Kamran
Professor, Pathology Department, Transplant Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Associate Professor, Gastroenterohepatology Research Center, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.
Middle East J Dig Dis. 2012 Jul;4(3):180-2.
Castleman's disease is an uncommon cause of lymph node enlargement that mostly involves the mediastinum and lung hilum. It is divided into 2 types: localized, which is usually asymptomatic and presents with a mass lesion; and multicentric, which is characterized by chills, anemia, generalized lymphadenopathy and hepatosplenomegaly. An extrathoracic site of involvement is very uncommon, and may be located in the mesentery of the intestines, axilla, and pelvis. Hepatic localization of this disease is a rare event. Herein, we report our experience with a symptomatic case of Castleman's disease in the porta hepatis who has been treated successfully by excision of the hilar lymph nodes, but recurred after 2 years with the same clinical picture plus abnormal liver function tests. However, at the time of recurrence ,no lymphadenopathy was detected and liver biopsy showed giant lymphoid follicles with germinal centers. She was treated with steroids and showed a dramatic response.
卡斯特尔曼病是淋巴结肿大的一种罕见病因，主要累及纵隔和肺门。它分为两种类型：局限性，通常无症状，表现为肿块病变；多中心性，其特征为寒战、贫血、全身淋巴结肿大和肝脾肿大。胸外受累部位非常罕见，可能位于肠系膜、腋窝和骨盆。这种疾病的肝脏定位是罕见事件。在此，我们报告了一例肝门部有症状的卡斯特尔曼病病例的经验，该病例通过切除肝门淋巴结成功治疗，但2年后复发，临床表现相同且肝功能检查异常。然而，复发时未检测到淋巴结病，肝活检显示有生发中心的巨大淋巴滤泡。她接受了类固醇治疗，反应显著。
