Anti-inflammatory effect of recombinant thrombomodulin for fulminant hepatic failure

Fulminant hepatic failure(FHF) is a critical illness that can be comorbid to primary liver damage.FHF shows a high mortality rate,and patients with FHF require intensive therapy,including plasma apheresis.However,intensive care at the present is not enough to restore the severe liver damage or promote hepatocellular reproduction,and a standard therapy for the treatment of FHF has not been established.An 86-year-old female with FHF was admitted to our hospital.Her manifestation demonstrated a clinical situation of systemic inflammatory response syndrome(SIRS) and disseminated intravascular coagulation.A diagnosis of fulminant hepatitis was made according to the definition given in the position paper of the American Association for the Study of Liver Diseases.Her serum hepatocyte growth factor(HGF) level had increased to 11.84 ng/m L.The HGF level indicated massive liver damage as seen in FHF.Recombinant thrombomodulin(r TM) was administered daily from the admission day for 1 wk at 380 U/kg.The patient's white blood cells and C-reactive protein responded to the r TM treatment within a few days.The HGF level and PT recovered to the normal range.The levels of proinflammatory cytokines(tumor necrosis factor-α and interleukin-1β) were suppressed by the administration of r TM.The patient's hepatic function(e.g.,PT and albumin) completely recovered without plasma exchange.r TM may modulate the over-response of SIRS with the improvement of proinflammatory cytokines.The underlying mechanism is thought to be the inhibitory effect of r TM on highmobility group box 1(HMBG1).The pathogenesis of HMBG1 protein in fulminant hepatic failure has beenalready known.A novel favorable effect of r TM for SIRS would be promising for FHF,and the wide application of r TM for SIRS should be considered.

肝移植后的继发急性髓系白血病

背景:肝移植后急性髓系白血病是一种极少见但病死率极高的并发症。目的:分析肝移植后急性髓系白血病的临床特征。方法:报告1例肝移植后发生急性早幼粒细胞性白血病病例,并辅以文献复习。结果与结论:患者接受原位肝移植后85个月,拔牙后出血不止,进一步查凝血功能异常、血常规、骨髓细胞学、白血病免疫分型检查提示为急性早幼粒细胞白血病、PML/RARa融合基因阳性,明确诊断急性早幼粒细胞性白血病,合并弥散性血管内凝血,给予输注新鲜冰冻血浆纠正凝血异常,经维甲酸、亚砷酸、柔红霉素联合诱导化疗,骨髓完全缓解。予柔红霉素和阿糖胞苷,米托蒽醌和阿糖胞苷巩固化疗2个疗程,之后定期予亚砷酸化疗,骨髓持续缓解。化疗同时,调整免疫抑制剂剂量和类型,患者肝功能稳定,未发生严重感染等并发症。结果说明,肝移植后可并发急性髓系白血病,以急性早幼粒细胞白血病常见,肝移植患者出现血象、凝血异常,要考虑急性早幼粒细胞白血病可能。应早期诊断及治疗,以减少死亡率。

PLASMIC积分对血栓性血小板减少性紫癜和弥散性血管内凝血鉴别的临床价值

目的比较PLASMIC积分在血栓性血小板减少性紫癜（TTP）和弥散性血管内凝血（DIC）患者中的差异，评价该积分系统对于两种疾病鉴别诊断的临床价值。方法回顾性分析24例TTP和41例DIC患者的临床资料，以及血小板计数、平均红细胞体积、间接胆红素、肌酐和凝血酶原时间．国际标准化比值，统计PLASMIC积分。结果根据PLASMIC积分预测伴有ADAMTSl3严重缺乏的TTP的危险度分层，24例TTP患者中，3例（12．5％）存在中度风险，21例（87．5％）存在高度风险。而在41例DIC患者中，38例（92．7％）存在低度风险，2例（4．9％）存在中度风险，仅有1例（2．4％）患者存在高度风险。在65例患者中，PLASMIC积分达高度风险对于TTP诊断的敏感性为87．5％，特异性为97．6％。结论PLASMIC积分系统高度风险的患者诊断为TTP的可能性大。该积分系统对于TTP和DIC的鉴别诊断有较高的临床价值。