Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học General Psychiatry cung cấp cho các bạn kiến thức về ngành y đề tài: Pediatric Hemophagocytic Syndromes: A Diagnostic and Therapeutic Challenge. | 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 Pediatric hemophagocytic syndrome HS is a distinct clinical entity in which excessive uncontrolled activation and proliferation of T cells and macrophages occur and are often fatal. First described in 1939 by Scott and Robb-Smith as a histiocytic reticulosis a neoplastic proliferation of histiocytes 1 this syndrome has since then been given several other denominations including N. Jabado Division of Haematology and Oncology Department of Paediatrics Montreal Children s Hospital McGill University Health Centre Montreal Quebec C. McCusker Division of Allergy and Immunology Department of Paediatrics Montreal Children s Hospital McGill University Health Centre Montreal Quebec G. de Saint Basile INSERM U429 Hôpital Necker Enfants-Malades 149 rue de Sèvres 75015 Paris France Correspondence to Dr. Nada Jabado Division of Haematology and Oncology Department of Paediatrics Montreal Children s Hospital McGill University Health Centre Montreal PQ H3Z 2Z3 E-mail N. Jabado and C. McCusker are recipients of a Chercheur Boursier Award from Fondation de la Recherche en Sante au Quebec hemophagocytic histiocytosis histiocytic disorder macrophage activation syndrome and reactive hemophagocytic lymphohistiocytosis HLH .2 3 To date this syndrome remains ill-recognized in children leading to false or .
