噬血细胞综合征（HLH）被认为是一种单核巨噬系统 反应性 增生的 组织细胞 病，主要是由于 细胞毒 杀伤细胞 （CTL）及 NK细胞 功能缺陷 导致抗原清除障碍，单核巨噬系统接受持续抗原刺激而过度活化增殖，产生大量 炎症细胞因子 而导致的一组临床综合征。 噬血细胞综合征主要表现为 发热 、 脾大 、 全血细胞减少 、高甘油三酯、低 纤维蛋白原 、高 血清铁蛋白，并可在骨髓、脾脏或 淋巴结活检 中发现噬血现象。 噬血细胞综合征主要分为 原发性 （遗传性）及继发性。 前者 …

In hematology, hemophagocytic lymphohistiocytosis (HLH), also known as haemophagocytic lymphohistiocytosis (British spelling), and hemophagocytic or haemophagocytic syndrome, [1] is an uncommon hematologic disorder seen more often in children than in adults.

Hemophagocytic lymphohistiocytosis is a rare and often life-threatening condition if left untreated. HLH causes your immune system to attack your body instead of a foreign invader like a virus. The condition is either acquired or genetic and there’s no way to prevent it. Treatment is available and most effective with an early diagnosis.

噬血细胞性淋巴组织细胞增多症（HLH）是一种罕见的疾病，主要引起婴儿和幼儿的免疫功能障碍。 尽管大多数患者不能明确原发病，但许多患者有原发性免疫性疾病。 临床表现可能包括淋巴结肿大、肝脾肿大、发热及神经系统异常。 通过特定的临床表现和实验室（遗传学）检查可明确诊断。 常予化疗，而在难治性或有遗传因素参与致病的患者中，可进行造血干细胞移植。 (参见组织细胞病概述。 噬血细胞性淋巴组织细胞增多症（HLH）较少见它主要影响<18 个月的婴儿，但 …

2016年6月10日 · 噬血细胞综合征又称噬血细胞性淋巴组织细胞增生症（hemophagocytic lymphohistiocytosis， HLH），是一组由多种病因诱发细胞因子「瀑布式」释放，组织病理学以组织细胞增生伴随其吞噬各种造血细胞为特征的综合征。 FHL 的遗传模式为常染色体隐性遗传，如果患者为第一胎或者没有患病的兄弟姐妹，多数医生很难确诊该病。 因此，现有的统计学数据中 HLH 的发病率或许被低估了。 中国 HLH 的发病率目前没有明确数据。 继发性 HLH（acquired …

2024年11月7日 · Hemophagocytic lymphohistiocytosis (HLH) is a rapidly progressing, life-threatening syndrome characterized by excessive immune activation, often presenting as a complex cytokine storm. This hyperactive immune response can lead to multi-organ failure and systemic damage, resulting in an extremely short survival period if left untreated.

Hemophagocytic lymphohistiocytosis (HLH) covers a wide array of related life-threatening conditions featuring ineffective immunity characterized by an uncontrolled hyperinflammatory response. HLH is often triggered by infection.

2024年6月5日 · HLH causes a hemorrhagic form of DIC with increased risk of bleeding due to low fibrinogen levels. Prior to lumbar puncture, consider checking a fibrinogen level, coagulation studies, and complete blood count. Basic chemistry findings may include elevated protein levels and lymphocytic pleocytosis.

2015年12月5日 · Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by extreme immune activation, resulting in pathologic inflammation. The diagnosis includes a spectrum of inherited or acquired defects in cytotoxic …

2022年12月15日 · Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome due to aberrant activation of macrophages. It can be challenging to diagnose & often leads to critical illness with multiple organ system failure. This ICU OnePager reviews the etiology, diagnostic criteria, and treatment approach to this complex illness.