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  • Hemophagocytic lymphohistiocytosis: an update on diagnosis and pathogenesis.

Hemophagocytic lymphohistiocytosis: an update on diagnosis and pathogenesis.

American journal of clinical pathology (2013-05-22)
Flavia G N Rosado, Annette S Kim
摘要

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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Sigma-Aldrich
铁蛋白 来源于马脾脏, Type I, saline solution
Sigma-Aldrich
铁蛋白 来源于人类脾脏, Type V, 10 μg/mL in 0.15 M NaCl, 10 mM Tris, pH 8.0, containing 0.1% sodium azide
Sigma-Aldrich
铁蛋白 来源于人类肝脏, Type IV, 10 μg/mL