Acute fulminant hemolysis after transcatheter mitral valve replacement for mitral annular calcification

Transcatheter mitral valve replacement (TMVR) is emerging as an alternative treatment strategy to surgery for patients with severe mitral annular calcification (MAC) who are not candidates for traditional mitral valve surgery. Paravalvular leak (PVL) is common following TMVR for severe MAC and can lead to heart failure symptoms and/or intravascular hemolysis, the latter of which usually is clinically stable. We report the case of a 67‐year‐old woman with symptomatic severe aortic stenosis and mitral stenosis with MAC in the setting of prior chest irradiation who was treated initially with transcatheter aortic valve replacement followed by TMVR at a later date (Sapien S3 system; Edwards Lifesciences). Immediately following TMVR, she developed acute profound hemolysis which manifested with hemoglobinuria, transfusion‐dependent anemia, and acute renal failure requiring renal replacement therapy. She was treated with post‐dilation balloon valvuloplasty after failed transcatheter PVL closure 10 days following TMVR with resulting improvement in the PVL. The hemolytic anemia resolved and renal function recovered without the need for continued hemodialysis 2 months later and stabilization of glomerular filtration rate at 6 months. This case highlights a potential severe complication of TMVR in MAC and suggests that improvement in hemolysis and late recovery of renal function may occur following treatment of PVL.     

Predictive mathematical models of cancer signalling pathways

Complex intracellular signalling networks integrate extracellular signals and convert them into cellular responses. In cancer cells, the tightly regulated and fine-tuned dynamics of information processing in signalling networks is altered, leading to uncontrolled cell proliferation, survival and migration. Systems biology combines mathematical modelling with comprehensive, quantitative, time-resolved data and is most advanced in addressing dynamic properties of intracellular signalling networks. Here, we introduce different modelling approaches and their application to medical systems biology, focusing on the identifiability of parameters in ordinary differential equation models and their importance in network modelling to predict cellular decisions. Two related examples are given, which include processing of ligand-encoded information and dual feedback regulation in erythropoietin (Epo) receptor signalling. Finally, we review the current understanding of how systems biology could foster the development of new treatment strategies in the context of lung cancer and anaemia.     

“魔鬼周”极限训练对武警特战队员睾酮、心肌酶谱及血液分析的影响

 目的 了解“魔鬼周”极限训练对武警特战队员身体状况的影响,以便更有效地预防和减少运动损伤,提高体能训练效果。方法 选取79例来自武警某部参加2017年第三季度“魔鬼周” 极限训练的特战队员作为研究对象,分别于“魔鬼周”的第1天及第7天进行相关血液指标的检测,包括睾酮(T)、天门冬氨酸氨基转移酶(AST)、乳酸脱氢酶 (LDH)、肌酸激酶(CK)、肌酸激酶MB型同工酶(CK-MB)、白细胞数目(WBC)、中性粒细胞百分比(N)、淋巴细胞百分比(L)、红细胞数目(RBC)、血红蛋白浓度(HB)、血小板数目(PLT)。应用SPSS 18.0统计软件进行数据处理。结果 参加“魔鬼周”极限训练的武警特战队员训练后的血清AST、LDH、CK、CK-MB分别为(46.15±43.21)U/L、(285.14±62.76)U/L、(505.17±359.73)U/L、(26.42±16.03)U/L,较训练前(19.67±4.05)U/L、(214.38±31.84)U/L、(222.50±116.09)U/L、(15.85±3.02)U/L明显升高,差异有统计学意义(P＜0.05),T、WBC、L、RBC、HB、PLT低于训练前[(21.10±5.58)nmol/L、(5.64±1.24)×10⁹/L、(34.53±7.10)%、(4.82±0.34)×10¹²/L 、(147.20±7.58)g/L、(216.53±45.35)×10⁹/L vs(22.41±5.00)nmol/L、(6.00±1.42)×10⁹/L、(36.01±8.15)%、(4.93±0.35)×10¹²/L、(150.75±7.30)g/L、(258.96±47.26)×10⁹/L,P＜0.05],N训练前后无明显变化[(57.19±7.26)% vs (56.66±8.62)%]。结论 “魔鬼周”极限训练对武警特战队员的生理功能可产生一定的影响,进而可能影响其训练水平。     

SYSMEX XN-1000血球仪在脑脊液和胸腹水白细胞计数中的可行性探讨

目的：探讨SYSMEX XN-1000血液分析仪(简称SYSMEX XN-1000)的体液模式在脑脊液和胸腹水白细胞计数中应用的可行性。方法分别采用手工显微镜镜检法和SYSMEX XN-1000对183例脑脊液和胸腹水标本进行白细胞计数,将所测结果按A：(0~1000)×106/L,B：(1001~5000)×106/L,C：>5000×106/L分为三组,分别进行统计学分析。结果与手工显微镜镜检法相比,SYSMEX XN-1000所测结果差异无统计学意义(P>0.05)。A、B、C三组的相关系数分别为0.993、0.984、0.996,A组回归方程为y=1.007x-1.072,B组回归方程为y=1.003x+19.776,C组回归方程为y=1.241x-1419.365。结论 SYSMEX XN-1000血液分析仪可用于临床脑脊液和胸腹水中白细胞计数,其操作简单易行,结果稳定可靠,但如果仪器进行样品检测时报警,应当手工镜检复核,以提高结果的准确性和可靠性。     

腹水中高荧光强度细胞的检测分析

目的 探讨腹水中高荧光强度细胞(HF-BF)临床应用价值.方法 应用XT-4000i全血细胞分析仪体液模式分析760例肝硬化和肝癌患者腹水细胞中HF-BF绝对值和比率,分别比较其在肝硬化、肝癌组中的情况,以及不同的Child-Pugh分级、肝癌临床分期以及腹腔感染对结果的影响.结果 肝癌组和肝硬化组的HF-BF绝对值分别为0.031(0.011,0.082)×109/L、0.026(0.011,0.052)×109/L,比率分别为8.6.(2.90,25.70)％、6.90(3.00,11.90)％,肝癌组HF-BF绝对值和比率均明显高于肝硬化组,差异均有统计学意义(P=0.000,P=0.022).且不同Child-Pugh分级的肝癌患者HF-BF绝对值和比率均有统计学意义(P=0.000).而不同Child-Pugh分级的肝硬化患者中仅HF-BF比率的差异有统计学意义(P=0.014).肝硬化腹水合并腹腔感染患者的HF-BF的比率和绝对值显著高于无腹腔感染患者(P=0.003,P=0.009),肝癌组合并腹腔感染患者的HF-BF的比率显著高于无腹腔感染患者(P=0.040).肝硬化合并腹腔感染患者和肝癌组合并腹腔感染患者腹水的HF-BF的比率和绝对值间差异无统计学意义(P＞0.05).不同临床分期的肝癌患者HF-BF的比率和绝对值的差异无统计学意义(P＞0.05).结论 通过体液模式HF-BF的分析,有助于对腹水良恶性的判断,同时提示患者是否有腹腔感染,其中HF-BF的比率意义更明显.     

中性粒细胞缺乏血液病患者并发嗜麦芽窄食单胞菌败血症的临床特征和预后分析

目的 探讨中性粒细胞缺乏血液病患者发生嗜麦芽窄食单胞菌败血症的临床特征和预后因素。方法 回顾性分析我科2006年1月—2016年12月收治的32例发生嗜麦芽窄食单胞菌败血症合并中性粒细胞缺乏患者的临床资料。结果 32例患者中急性白血病20例, 重型再生障碍性贫血 （SAA） 7例, 非霍奇金淋巴瘤5例。所有患者长时间应用广谱抗生素, 中心静脉置管 （21例） 或者外周静脉置管 （11例）。25例化疗后出现中性粒细胞缺乏, 7例SAA 为本病导致中性粒细胞缺乏。死亡17例, 好转15例。药敏试验显示多药耐药, 替加环素、 甲氧苄啶-磺胺甲噁唑、 左氧氟沙星、 头孢哌酮/舒巴坦、 头孢他啶、 哌拉西林/他唑巴坦部分敏感, 其余均耐药。重度中性粒细胞缺乏、 重度血小板减少、 粒细胞减少持续时间过长和复合感染等是预后不良因素。结论 中性粒细胞缺乏血液病患者并发嗜麦芽窄食单胞菌败血症死亡率高, 及时诊断和治疗对预后至关重要。     

EML4::ALK fusions in complex lymphatic malformations

Gorham–Stout disease (GSD) and generalized lymphatic anomaly (GLA) are subtypes of complex lymphatic malformations (CLMs) with osseous involvement that cause significant complications, including pain and pathologic fractures. As with other vascular anomalies, somatic mosaic mutations in oncogenes are often present, and the mTOR inhibitor sirolimus alleviates symptoms in some, but not all, patients. We describe two patients, one with GSD and one with GLA, found to have EML4::ALK fusions. This report of a targetable, oncogenic fusion in vascular malformations expands our understanding of the genetic basis for CLMs and suggests additional targeted therapies could be effective.     

Sysmex XN-9000血液分析仪前体白细胞通道（WPC）原始/异常淋巴细胞报警可靠性的评价

目的评价Sysmex XN-9000血液分析仪(简称"XN-9000")前体白细胞通道(WPC)检测外周血触发WBC报警的可靠性。方法采用与手工血涂片白细胞形态镜检结果比对的方法,复核520例分别经Sysmex XE-2100血液分析仪(简称"XE-2100")和XN-9000检测触发WBC报警的标本,按报警种类统计判读结果。结果 XN-9000 WPC通道的Blast(原始细胞)报警比XE-2100特异性高(χ~2=6.98,P0.05),XN9000将假阳性率从XN2100的64%降低至36%(χ~2=6.18,P0.05)。XN-9000 WPC的Blast/Abn Lympho(原始/异常淋巴细胞)综合有效率90.4%、94.4%,高于XE2100的72.2%和80.1%(χ~2分别为5.6、6.33,P均0.05)。在WPC通道自动复测模式下,在所有被检标本(体验健康者和患者,包括新生儿)中,WPC通道Blast/Abn Lympho(原始/异常淋巴)报警的特异性和总有效率高于WDF(χ~2=6.15、4.14、6.02、4.88,P0.05),降低了WDF通道误报率;XN-9000(WDF/WPC)总报警复片率低于XE-2100(χ~2=4.33,P0.05);而XN-9000(WDF/WPC)Blast/Abn Lympho(原始/异常淋巴)报警的复片率为32%(84/260),低于XE-2100的41%(107/260)(χ~2=4.38,P0.05)。结论XN-9000 WPC通道相较于XE-2100,提高了Blast/Abn Lympho(原始/异常淋巴)报警的综合有效性,降低了误报率和复片率,提高了临床实验室的工作效率。