
Hemophagocytic Lymphohistiocytosis: Symptoms, Causes & Outlook
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.
Hemophagocytic Lymphohistiocystosis - Johns Hopkins Medicine
Hemophagocytic lymphohistiocytosis (HLH) is a rare disease that usually occurs in infants and young children. It may also occur in adults. Children usually inherit the disease. In adults, many different conditions, including infections and cancer, can cause HLH.
Hemophagocytic lymphohistiocytosis - Wikipedia
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.
噬血细胞综合征 - 百度百科
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 …
Lymphohistiocytosis - StatPearls - NCBI Bookshelf
2023年3月27日 · Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening, hyper-inflammatory disorder affecting both newborns and adults alike. The disease is characterized by dysregulated immune activity resulting in malignant inflammation and multi-organ failure.
UpToDate
Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome of excessive immune activation. It most frequently affects infants from birth to 18 months of age, but the disease is also observed in children and adults of all ages.
噬血细胞综合征 - 知乎 - 知乎专栏
噬血细胞综合征 (hemophagocytic syndrome, HPS)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”释放,组织病理学检查可见组织细胞增生伴吞噬各种造血细胞为特征的临床综合征。 其临床特点是持续高热,伴肝脾、淋巴结肿大,肝功能严重受损,血二系或全血细胞减少,凝血功能异常等,可伴中枢神经系统受累。 【病因及分类】 HLH按病因可分为原发性和继发性两大类。 原发性又包括家族 …
噬血细胞性淋巴组织细胞增多症(HLH) - 血液病学及肿瘤病学
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 …
Hemophagocytic Lymphohistiocytosis | New England Journal of …
2025年2月5日 · Hemophagocytic lymphohistiocytosis (HLH) is a severe and life-threatening syndrome characterized by overwhelming inflammation that often leads to multiorgan failure and death if not treated ...
Hemophagocytic lymphohistiocytosis: current treatment advances ...
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.