pediatric hemophagocytic syndromes a diagnostic and therapeutic challenge儿科hemophagocytic综合征诊断和治疗的挑战.pdfVIP

Review Pediatric Hemophagocytic Syndromes: A Diagnostic and Therapeutic Challenge Nada Jabado, MD, PhD; Christine McCusker, MD; Genevieve de Saint Basile, MD, PhD Abstract Pediatric hemophagocytic syndrome (HS) is a severe and often fatal clinical disorder. This syndrome is frequently unrecognized, and thus, affected children may receive suboptimal management, leading to an increase in mortality. The purpose of this review is to provide a clinical guide to (1) the recognition of HS based on clinical, biologic, and pathologic features; (2) the identification of the primary cause of HS in a given affected child; and (3) the initiation of effective treatment in a timely manner. Background hemophagocytic histiocytosis, histiocytic disorder, macrophage activation syndrome, and reactive Pediatric hemophagocytic syndrome (HS) is a hemophagocytic lymphohistiocytosis (HLH).2,3 distinct clinical entity in which excessive To date, this syndrome remains ill-recognized in uncontrolled activation and proliferation of T cells children, leading to false or delayed diagnosis and macrophages occur and are often fatal. First and suboptimal management. Etiologically, HS is described in 1939 by Scott and Robb-Smith as a a component of several inherited disorders in histiocytic reticulosis, a neoplastic proliferation of which it is present at onset or during the course of histiocytes,1 this syndrome has since then been the disease. It has also been associated with a given several other denominations, including variety of viral, bacterial, fungal, and parasitic infections, as well as with collagen-vascular diseases4–6 and m