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2025年2月5日 · Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of overwhelming inflammation (hyperinflammation) that causes multiorgan failure and death, making...

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.

It is a life-threatening disease of severe hyperinflammation caused by uncontrolled proliferation of benign lymphocytes and macrophages that secrete high amounts of inflammatory cytokines. It is classified as one of the cytokine storm syndromes. There are inherited and non-inherited (acquired) causes of HLH.

Hemophagocytic lymphohistiocytosis (HLH) is a rare immune system disease. It occurs more often in babies and young children. But it can also occur in adults. Children may inherit the disease through genes. This is called familial HLH. But in adults, many conditions can cause HLH. This is called acquired HLH.

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.

噬血细胞综合征（HPS）又称为噬血细胞性淋巴组织细胞增多症（HLH），是一种遗传性或获得性免疫调节功能异常导致的严重炎症反应综合征。 近年来HLH的诊治策略不断更新，为更好地指导我国医师的临床实践，基于当前的循证医学证据，经多领域医学专家共同商讨，对2018年版的《噬血细胞综合征诊治中国专家共识》进行修订，制定《中国噬血细胞综合征诊断与治疗指南（2022年版）》。 本指南制定了HLH临床诊断和治疗路径，旨在进一步规范我国HLH的诊断和治疗。 引 …

Hemophagocytic lymphohistiocytosis (HLH) is an uncommon disorder causing immune dysfunction primarily in infants and young children. Many patients have an underlying immune disorder, although in some patients the underlying disorder is not known. Manifestations may include lymphadenopathy, hepatosplenomegaly, fever, and neurologic abnormalities.

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）是一种罕见病，通常发生在18个月以下的婴儿。 hlh可以是家族性的（遗传的）或获得性的。 如果患者符合8个已发布的诊断标准中的至少5个，或者有已知的与hlh相关的突变，可诊断hlh。