期刊
AMERICAN JOURNAL OF CLINICAL PATHOLOGY
卷 139, 期 6, 页码 713-727出版社
OXFORD UNIV PRESS INC
DOI: 10.1309/AJCP4ZDKJ4ICOUAT
关键词
Hemophagocytic lymphohistiocytosis; Hypercytokinemia; Acquired; Genetic; Perform; Macrophage activation syndrome
类别
Hemophagocytic lymphohistiocytosis (HLH) is a frequently fatal and likely underdiagnosed disease involving a final common pathway of hypercytokinemia, which can result in end-organ damage and death. Although an early diagnosis is crucial to decrease mortality, the definitive diagnosis is often challenging because of the lack of specificity of currently accepted diagnostic criteria and the absence of confirmatory gold standards. Because of the wide range of laboratory assays involved in the diagnosis of HLH, practicing pathologists from a broad spectrum of clinical specialties need to be aware of the disease so that they may appropriately flag results and convey them to their clinical counterparts. Our article summarizes these new advances in the diagnosis of HLH and includes a review of clinical findings, updated understanding of the pathogenesis, and promising new testing methods.
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