Hemophagocytic lymphohistiocytosis associated with HBV‐HCV coinfection in adult: Case report

The case reported is the first case in Morocco to our knowledge. The reason for sharing this case is to facilitate knowledge transfer between physicians, caring for adult patients with HLH, with the aim to improve the outcome of these patients.     

噬血细胞综合征患者血清sHLA-G表达及其临床意义

本研究旨在探讨血清可溶性人类白细胞抗原G(sHLA-G)在噬血细胞综合征(HPS)患者中的表达水平及临床意义。收集2008年9月至2010年7月经首都医科大学附属北京友谊医院确诊的HPS患者23例,根据不同病因分为感染相关性HPS、肿瘤相关性HPS和风湿免疫病相关性HPS,同时收集25例健康人血清,分别采用酶联免疫吸附(ELISA)法检测患者及健康人血清sHLA-G水平,并与各实验室检查指标进行相关性分析。结果表明,HPS患者组血清sHLA-G水平高于健康对照者,差异有显著统计学意义(p=0.003),对HPS患者组中不同病因人群进行单因素方差分析,差异无统计学意义(p=0.233)。检测HPS患者血清sHLA-G水平与采血当日WBC、Hb、Plt、ALT、AST、LDH、ALB、TBil、DBil、IBil、Cr、BUN、TG、纤维蛋白原、铁蛋白的相关性发现,其与血小板水平呈正相关关系,与其他各项实验室指标均无相关性。结论:血清sHLA-G水平在HPS患者中升高可能有多种原因,包括感染、肿瘤、T淋巴细胞过度活化以及一些细胞因子的刺激等,sHLA-G还可以抑制NK细胞活性,导致异常免疫风暴形成,在HPS的发病...     

腹部嗜血细胞综合征1例

患者女,54岁.10个月前无明显诱因出现发热,经药物治疗后症状无明显改善,并出现皮肤瘙痒,在当地医院应用激素治疗后好转;近2个月再次出现发热,伴上腹部不适、恶心、呕吐等.入院查体未见异常.实验室检查;血清铁蛋白897.00μg/L;RBC 3.48×1012/L;WBC 3.28×109/L;PLT 62.00×109/L.骨髓穿刺细胞涂片;组织细胞比例增高,可见巨噬细胞及吞噬血细胞现象.     

Recombinant activated factor VII in hemophagocytic lymphohistiocytosis with disseminated intravascular coagulation

Hemophagocytic lymphohistiocytosis (HLH) is a lifethreatening disorder due to hyperinflammation resulting in infiltration of different organs with extensive hemophagocytosis. Severe coagulopathy was one of the main reasons for death in HLH. Over secretion of plasminogen activator by activated macrophages leads to hyperfibrinolysis. We reported a 36-year-old woman who was diagnosed as HLH probably secondary to lymphoma. Massive bleeding from gut and retroperitoneal area were not able to be controlled by conventional hemostatic treatments. This patient received one dose recombinant activated factor VII (rFVIIa) 3.6 mg (70 µg/kg). Hemostatic effect was achieved in 0.5 hour and lasted 24 hours. Prothrombin time (PT) and activated partial thromboplastin time (APTT) were quickly corrected to normal ranges. Fibrinogen level elevated from 0.5 g/L before using rFVIIa to 1.8 g/L 20 hours after. Although dexamethasone and etopside were administrated to treat HLH, this patient died from septic shock after persistent neutropenia. This suggests that rFVIIa may be effective in the management of intractable hemorrhage in patients with HLH.

     

小儿噬血细胞综合征临床特点及预后危险因素分析

目的 分析小儿噬血细胞综合征(HPS)的临床特点及影响其预后的临床危险因素.方法 回顾性分析2007年1月～2010年12月住院治疗的32例HPS患儿的临床症状、体征和实验室检查特点,采用Logistic 回归分析方法,分析影响患儿预后的临床危险因素.结果 从性别、年龄、热程、病原学、外周血白细胞计数、血小板计数、血红蛋白、乳酸脱氢酶、谷丙转氨酶、单纯天冬氨酸转移酶、三酰甘油、纤维蛋白原、C-反应蛋白、活化部分凝血活酶时间中筛选出血小板降低(OR=2.078,P＜0.05)和EB病毒感染(OR=2.662,P＜0.05)为HPS死亡的高危因素.结论 EB病毒感染和血小板明显降低是儿童噬血细胞综合征预后不良的高危因素.该病预后凶险,死亡率高,应早期积极治疗,降低死亡率.