Trenn G, Gabbert H E, Schmitt-Gräff A, Reis H E
Krankenhaus Maria Hilf, Med. Klinik 1, Mönchengladbach.
Z Gastroenterol. 1999 Feb;37(2):159-63.
Sarcoidosis is a chronic multisystem disorder of unknown cause characterized by the presence of noncaseating epitheloid granulomas and derangement of the normal skin architecture. Though an array of organs may be affected by the disease the most common site of affection is the lung. An extrathoratic manifestation is rare. We describe a 66-year-old patient who was admitted to our hospital because of weight loss and hepatomegaly. A thorough examination revealed the diagnosis of a granulomatous hepatitis characterized by a markedly elevated alkaline phosphatase concentration of 1,490 U/I. A drug-induced hepatitis could be excluded and no evidence was found for the existence of a bacterial or viral infection or an autoimmune disorder. An ERCP revealed a normal common bile duct and normally branching small intrahepatic ducts. The patient was discharged with the diagnosis of a biliary cirrhosis. Half a year later the patient was readmitted to the hospital because of severe intestinal bleeding due to pancytopenia. A bone marrow biopsy showed infiltration of the marrow by granulomas. A histiocytosis X could be ruled out. The diagnosis of an extrathoracic sarcoidosis was assumed and a therapy with prednisone was started. Within six weeks the blood count normalized. After 18 months the serum alkaline phospatase concentration also normalized and no granulomas were found in the bone marrow. The case demonstrates that pancytopenia in sarcoidosis is not due to bone marrow failure.
结节病是一种病因不明的慢性多系统疾病，其特征为出现非干酪样上皮样肉芽肿以及正常皮肤结构紊乱。尽管该疾病可累及一系列器官，但最常见的受累部位是肺。胸外表现较为罕见。我们描述了一名66岁因体重减轻和肝肿大入院的患者。全面检查后诊断为肉芽肿性肝炎，其碱性磷酸酶浓度显著升高，达1490 U/I。可排除药物性肝炎，未发现存在细菌或病毒感染或自身免疫性疾病的证据。内镜逆行胰胆管造影（ERCP）显示胆总管正常，肝内小胆管分支正常。患者出院时诊断为胆汁性肝硬化。半年后，患者因全血细胞减少导致严重肠道出血再次入院。骨髓活检显示骨髓有肉芽肿浸润。可排除组织细胞增多症X。考虑诊断为胸外结节病，并开始使用泼尼松治疗。六周内血细胞计数恢复正常。18个月后血清碱性磷酸酶浓度也恢复正常，骨髓中未发现肉芽肿。该病例表明结节病中的全血细胞减少并非由于骨髓衰竭。
