标题:
继发性噬血性淋巴组织细胞增生症诊断和治疗新进展
Recent Advances in Diagnosis and
Treatment of Secondary
Hemophagocytic Lymphohistiocytosis
作者:
欧丹艳, 罗建明, 唐利娟
关键字:
噬血性淋巴组织细胞增生症, 继发性, 发病机制, 诊断, 治疗Hemophagocytic Lymphohistiocytosis, Secondary, Pathogenesis, Diagnosis, Treatment
期刊名称:
《Medical Diagnosis》, Vol.4 No.1, 2014-06-11
摘要:
噬血性淋巴组织细胞增生症(Hemophagocytic Lymphohistiocytosis, HLH)是一种少见的威胁生命的疾病，它是由过度增生的活化淋巴细胞和巨噬细胞产生大量细胞因子所造成的过度炎症反应。近年来在HLH的诊断和治疗上取得了一定的进展，但重症HLH的死亡率依然较高。HLH一般分为原发性和继发性。与原发性HLH相比，继发性HLH更常见，病因复杂，预后差异较大。本文对继发性HLH的诊断与治疗研究现状作一综述。
Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal syndrome. It is associated with uncontrolled proliferation of activated lymphocytes and macrophages which secrete great amounts of inflammatory cytokines. Despite progress in diagnosis and treatment, severe HLH still has a high mortality rate. HLH is classified as two subgroups: primary HLH and secondary HLH. Secondary HLH is involved in severe infections, malignancies and autoimmune diseases. This review seeks to update readers on current status and research progress related to this uncommon but life-threatening disease.