childrenliver diseaseCMV, cytomegalovirusEBV, Epstein Barr virusHLH, hemophagocytic lymphohistiocytosisLKM, liver–kidney-microsomalMAS, macrophage activation syndromePT, prothrombin timePTT, partial thromboplastin timeHemophagocytic lymphohistiocytosis (HLH) is a rare acute hyperinflammatory condition presenting with...
Although FHL is an autosomal recessive disorder typically occurring in infancy, it is important to clarify that the disease may also occur in older patients. It is now considered that FHL is a disorder of T-cell function; moreover, clonal proliferation of T lymphocytes is observed in a few ...
in ch il dr en w i th H L H -200 4 p r otocol Z H U Y in, GA0 Y i-j in,ZHU Xiao— hua,CHEN X iao— yun ( Children" s Hospital o f Fudan University ,Shanghai China) Abstract: Obj ective Hemophagocytic lymphohistiocytosis ( HLH) is a pediatr i c disease with ...
HLH)是 由于细胞毒T淋巴细胞(cytotoxic T-lymphocytes, CTLs)和NK细胞 毒效应显著降低和障碍,不能及时有效清除病毒等抗原(免疫 无效),巨噬细胞异常持续活化和增生所致的多器官高炎症反 应和组织器官免疫损伤的临床综合征 HLH起病急、病情进展迅速、病死率高,为一种潜在致死性疾 病 (potentially fatal disease)。
体(Notanindependentdiseaseentity),只是一种临床综合症,具有多种病因或基础疾病抗原持续刺激所致CTL和巨噬细胞高度活化但免疫无效、以细胞因子风暴为显著特征的高炎症反应综合征(highlystimulatedbutineffectiveimmuneresponsetoantigens,characterizedbylife-threateningcytokinestormandhyperinflammatoryreaction)TangYM,XuXJ.Advances...
lymphohistiocytosis,HLH)是由于细胞毒T淋巴细胞(cytotoxicT-lymphocytes,CTLs)和NK细胞毒效应显著降低和障碍,不能及时有效清除病毒等抗原(免疫无效),巨噬细胞异常持续活化和增生所致的多器官高炎症反应和组织器官免疫损伤的临床综合征HLH起病急、病情进展迅速、病死率高,为一种潜在致死性疾病(potentiallyfataldisease)。
As her condition deteriorated doctors diagnosed Layla with hemophagocytic lymphohistiocytosis (HLH), an immunodeficiency disease which, it is estimated, affects just one in 830,000 children a year. Tragic Layla struck down by disease just days after concert Thereby, he was provisionally diagnosed with...
具有不同树突状细胞表型的孤立性组织细胞瘤（solitaryhistiocytomasofvariousdendriticcellphenotypes）巨噬细胞相关性疾病（Macrophage-relateddisorders）原发性或继发性噬血细胞综合征（Hemophagocyticsyndrome，primaryorsecondary）罗-道病(Rosai-Dorfmandisease，RD)(淋巴结窦组织细胞增生症伴广泛淋巴结肿大，sinushistiocytosis...
, HLH)是由于细胞毒T淋巴细胞(cytotoxic T-lymphocytes, CTLs)和NK细胞毒效应显著降低和障碍,不能及时有效清除病毒等抗原(免疫无效),巨噬细胞异常持续活化和增生所致的多器官高炎症反应和组织器官免疫损伤的临床综合征 HLH起病急、病情进展迅速、病死率高,为一种潜在致死性疾病 (potentially fatal disease)。
2、疾病缓解(non-active disease):需达到以下标准。 体温正常; 脾脏大小恢复(少数病人可持续存在单纯性脾脏轻度肿大); 外周血象恢复(Hb≥90g/L,PLT≥100×109/L,ANC≥1.5×109/L); 甘油三酯水平恢复(<3mmol/L); 血清铁蛋白<500μg/L; 脑脊液正常(针对初诊时脑脊液阳性的病例); (对于有检测条件者,sCD25水...