以肝病为表现的儿童噬血细胞性淋巴组织细胞增生症（HLH）。
Hemophagocytic Lymphohistiocytosis (HLH) in Children Presenting as Liver Disease.
作者信息
Amin Nomisha, Shah Ira, Bhatnagar Sushmita
机构信息
Pediatric Liver Clinic, B.J. Wadia Hospital for Children, Mumbai, India.
出版信息
J Clin Exp Hepatol. 2014 Jun;4(2):175-7. doi: 10.1016/j.jceh.2013.06.003. Epub 2013 Jul 2.
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a rare acute hyperinflammatory condition presenting with high fever, pancytopenia, splenomegaly with the pathologic finding of hemophagocytic lymphohistiocytosis in bone marrow and other tissues. Predominant hepatic manifestations at presentation are rare. We report a series of three cases which showcase the spectrum of liver disease as presentation in hemophagocytic lymphohistiocytosis.
摘要
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的急性高炎症性疾病，表现为高热、全血细胞减少、脾肿大，骨髓和其他组织中存在噬血细胞性淋巴组织细胞增生症的病理表现。以肝脏表现为主的情况在发病时较为罕见。我们报告了一系列三例病例，展示了噬血细胞性淋巴组织细胞增生症以肝病为表现的疾病谱。
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