Jones M B, Voet R, Pagani J, Lotysch M, O'Connell T, Koretz R L
Gastroenterology. 1981 Feb;80(2):384-9.
The multifocal eosinophilic granuloma syndrome (Hand-Schüller-Christian disease) is characterized histologically by focal accumulations of histiocytes and eosinophils. The clinical manifestations of this syndrome reflect the site and extent of these focal accumulations which are usually found in membranous bone, skin, and lung. Significant hepatobiliary disease due to this disorder has not been previously described. We are reporting an adult female with the multifocal eosinophilic granuloma syndrome who developed a mixed histiocytic and eosinophilic infiltration of the common bile duct which resulted in obstructive jaundice. Therapeutic attempts have failed to reverse this process, and the patient has shown progressive intrahepatic biliary tract destruction.
多灶性嗜酸性肉芽肿综合征（汉-许-克病）在组织学上的特征是组织细胞和嗜酸性粒细胞的局灶性聚集。该综合征的临床表现反映了这些局灶性聚集的部位和范围，这些聚集通常见于膜性骨、皮肤和肺。此前尚未描述过该疾病导致的显著肝胆疾病。我们报告了一名患有多灶性嗜酸性肉芽肿综合征的成年女性，其胆总管出现了组织细胞和嗜酸性粒细胞的混合浸润，导致梗阻性黄疸。治疗尝试未能逆转这一过程，且患者出现了进行性肝内胆管破坏。
