白血病患者并发真菌肺炎行异基因移植7例临床分析

目的观察并发曲霉菌肺炎的白血病患者行异基因外周血造血干细胞移植的疗效及安全性。方法回顾性分析7例伴曲霉菌肺炎的白血病患者成功完成异基因外周血造血干细胞移植的过程。结果入组7例患者中,男4例,女3例,中位年龄22.8（13-37）岁,随访时间均大于6个月,移植后1个月复查患者嵌合体均为100%完全供者嵌合。移植后平均抗真菌治疗时间为3个月,7例患者肺部真菌感染全部达到显效,造血干细胞100%植入,无1例发生相关死亡。结论伴发曲霉菌肺炎的白血病患者采用有效的抗真菌治疗方案仍然可以接受异基因外周血造血干细胞移植,并不会影响供者造血干细胞植入,且患者耐受性良好,值得进一步研究。     

异基因造血干细胞移植后早期EB病毒激活的监测和抢先治疗

目的 研究异基因造血干细胞移植(allo-HSCT)后早期EB病毒(EBV)激活的监测及其抢先治疗的结果和预后.方法 以2007年1月至2009年1月在我院行allo-HSCT并连续监测血浆EBV DNA的277例患者为研究对象,其中亲缘人类白细胞抗原(HLA)单倍型移植116例;非血缘移植75例,同胞相合移植86例.预处理主要采用马利兰(BU)+环磷酰胺(CY)/氟达拉滨(Flu)或全身照射(TBI)/Flu方案,此外,亲缘单倍型和非血缘移植加用抗胸腺细胞球蛋白(ATG).移植后最初的3个月内每周采用实时定量PCR(RQ-PCR)方法检测血浆EBV DNA,若＞5×102拷贝/ml而无临床症状时诊断为EBV血症.抢先治疗主要应用阿昔洛韦10 mg/kg静脉点滴,每8小时1次,同时在情况允许时减少免疫抑制剂.结果 移植后100 d(+100 d)内33例(11.9%)患者发生EBV血症,中位时间为+44(+19～+84)d;EBV血症的发生率在同胞相合、亲缘单倍型和非血缘移植中分别为0、15.5%和20.0%,在亲缘单倍型与非血缘移植中EBV血症的发生率差异无统计学意义(P=0.09),但均较同胞相合移植高(P=0.001).33例患者均首先采用减少免疫抑制剂剂量和阿昔洛韦抢先抗病毒治疗,其中20例患者病毒血症转为阴性,治疗有效率为60.6%,中位时间为治疗后11(4～56)d,抗病毒治疗疗程的中位时间为21(14～60)d.单因素及多因素分析均表明,亲缘单倍型移植、非血缘移植以及Ⅱ～Ⅳ度急性移植物抗宿主病(aGVHD)是EBV血症发生的高危因素.发生EBV血症患者(33例)比未发生EBV血症患者(244例)的2年预期生存率明显降低(54.2%比72.1%,P=0.006).结论 allo-HSCT后用RQ-PCR监测血浆EBV DNA载量可以及时确定EBV血症并给予抢先抗病毒治疗,且大多数患者对抢先治疗有完全反应,这样有利于减少高危患者EBV相关疾病的发生率和病死率;亲缘单倍型移植、非血缘移植及Ⅱ～Ⅳ度aGVHD是EBV血症的高危因素;EBV血症对allo-HSCT后患者的生存有负面影响.

Abstract：

Objective To investigate early Epstein-Barr virus (EBV) reactivation and the outcome of preemptive therapy after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Methods From January 2007 to January 2009, totally 277 patients after allo-HSCT were studied (haploidentical 116,unrelated 75, matched sibling 86). Conditioning regimens were mainly busulfan (BU) + cyclophosphamide ( CY)/fludarabine(Flu) or total body irradiation (TBI) + CY/Flu. Antihuman thymocyte globulin (ATG)was added in haploidentical and unrelated transplants. Plasma EBV DNA was monitored once to twice weekly in the first 3 months after allo-HSCT with real time quantitative polymerase chain reaction (RQ-PCR). EBV viremia was diagnosed when EBV DNA was more than 5 × 102 copies/ml but without symptoms. Acyclovir (10 mg/kg, intravenous drip, 8 h) was used for preemptive therapy and immnuo-suppressants were decreased if possible. Results Totally 33 patients ( 11.9% ) developed EBV viremia with a median time at day 44 (day 19 to day 84). The incidences of EBV viremia in the transplants from matched sibling,haploidentical, unrelated donors were 0, 15.5%, 20. 0%, respectively. There was no significant difference between haploidentical and unrelated transplants ( P = 0. 09 ), but much less EBV viremia was seen in matched sibling transplant ( P = 0. 001 ). Twenty of 33 patients ( 60. 6% ) had complete response to preemptive therapy. The median time to reach EBV DNA negative in plasma was 11 (4-56) d. The median duration of preemptive therapy was 21 (14-60) d. Both univariate and multivariate analysis indicated that haploidentical and unrelated transplants, acute graft versus host disease (GVHD) were the risk factors for EBV viremia. Two-year overall survival in the patients with EBV viremia was significantly lower than that without EBV viremia (54. 2% vs 72. 1%, P = 0. 006 ). Conclusions Our large clinical study has demonstrated that preemptive therapy with acyclovir that is guided by EBV viremia is effective in majority of the patients with high-risk for EBV reactivation after allo-HSCT, which may further decrease the risk for developing life-threatening EBV disease or post-transplantation lymphoproliferative disorder. Haploidentical and unrelated transplants, acute GVHD are the risk factors for EBV viremia which has negative impact on survival.     

散发性包涵体肌炎临床病理分析

目的探讨散发性包涵体肌炎(sIBM)患者的临床及病理特点。方法收集2例于2008年至2010年就诊并明确诊断为s1BM的患者临床、病理资料。两例患者均有股四头肌无力和萎缩,1例出现肢体远端无力和上肢无力。2例患者均进行了肌肉活体组织检查标本的组织学、酶组织化学染色和免疫组织化学染色。结果 2例患者肌酶均轻度升高。肌电图检查示1例呈肌源性损害,1例呈神经源性损害。2例患者的骨骼肌主要病理改变都是肌内衣炎细胞浸润、肌纤维萎缩,肌纤维内嗜碱性镶边空泡。免疫组织化学染色提示CD8+淋巴细胞浸润为主,1例患者镶边空泡肌纤维内Ubiquitin染色阳性。结论本文2例sIBM以股四头肌损害明显,病情缓慢进展,依靠肌肉活检确定诊断。     

多中心型Castleman病伴嗜酸细胞升高1例报道并文献复习

目的了解Castleman病（Castleman＇s Disease,CD）的临床特征并提高诊断和治疗水平。方法报道1例以嗜酸细胞升高为主要表现的CD的临床资料并结合文献复习,为其诊断和鉴别诊断提供新思路。结果患者男性,57岁,体检发现嗜酸细胞增多;双侧颈部、腋窝、腹股沟及腹膜后多个淋巴结肿大;淋巴结活检病理：血管滤泡性淋巴结组织增生,浆细胞型。结论临床表现多样,组织病理学检查或淋巴结活检有益于早期诊断,最佳治疗效果基于组织病理学和临床分类。     

无功能肾上腺肿瘤82例临床分析

目的分析无功能肾上腺肿瘤的病理及临床特征。方法无功能肾上腺肿瘤82例,术前经B超(80例)、CT扫描(79例)或MRI(26例)定位诊断,81例经手术病理证实,1例晚期患者,未行手术死亡。所有患者术前各项内分泌指标检测结果均在正常范围。结果 81例手术患者,80例顺利切除肿瘤,术中未发现血压波动。80例术后随访1年以上未发现肿瘤复发。结论 B超、CT为无功能肾上腺肿瘤首选诊断方法;直径>4 cm肿瘤需手术切除。     

YD383和YD439对IL-4诱导的BJAB细胞CD23表达的影响

目的:初步探讨2种化合物YD383、YD439对JAK/STAT6信号传导通路的抑制机制。方法:将不同剂量的化合物YD383和YD439加入对数生长期的BJAB细胞作用30min,终浓度为20μg/L的白细胞介素(IL)-4共同孵育3h后,收集细胞并加入PE标记的鼠抗人CD23抗体,流式细胞仪检测各细胞群体CD23分子的表达情况。结果:化合物YD383和YD439均能降低由IL-4诱导的BJAB细胞CD23分子的表达,且随着化合物剂量的增大,CD23分子的表达水平逐渐降低。2种化合物各自不同的剂量组间CD23分子表达的差异均有统计学意义(P<0.05)。结论:化合物YD383和YD439对IL-4诱导的CD23分子的表达均有明显的抑制作用,并且具有剂量依赖性。     

恶性血液病合并肺部侵袭性真菌感染31例临床分析

目的探讨恶性血液病合并肺部侵袭性真菌感染（invasive fungal infection,IFI）的临床特点。方法对31例并发肺部IFI的恶性血液病患者的临床特征进行回顾性分析。结果广谱抗生素使用时间≥7d,IFI出现前中性粒细胞缺乏时间≥7d,中性粒细胞缺乏持续时间≥10d是并发IFI的重要诱因。感染菌种主要为白色念珠菌（占9.7%,3株）、非白色念珠菌（占12.9%,4株）和曲霉菌（29.1%,9株）。虽经抗真菌治疗,仍有8例患者死于呼吸衰竭。结论恶性血液病患者易于发生IFI,且一旦发生病死率明显增加,应及早进行预防和早期经验性抗真菌治疗。     

Splenic Injury Following Colonoscopy – An Underdiagnosed,But Soon to Increase,Phenomenon?

INTRODUCTION

We present a case of splenic rupture in a 71-year-old woman admitted 6 days following a diagnostic colonoscopy. She underwent an open splenectomy and made a delayed, but complete, recovery.We proceeded to perform a retrospective review of all relevant literature to assess the frequency of similar post-colonoscopy complications.

MATERIALS AND METHODS

Using relevant keywords, we identified 63 further PubMed reports of splenic injury associated with colonoscopy that were reported in English.

FINDINGS

We have described only the fourth report of splenic injury secondary to colonoscopy from a UK centre. Literature review reveals a mean age of 63 years and a female preponderance for this complication.Most patients present on the day of their colonoscopy with abdominal pain, anaemia, elevated white cell count and Kehr''s sign. CT is the investigation of choice and splenectomy the definitive management of choice. Most patients make a routine recovery, with mortality rates of approximately 8%.There is likely to be an under-reporting of this complication from UK-based centres,with the majority of reports originating from Europe and US.This points to a possible under-diagnosis or under-recognition of this potentially fatal complication. The incidence of such post-colonoscopic complications may increase with the forthcoming introduction of the National Bowel Cancer Screening Programme.     

主动脉夹层患者的心理社会因素分析

目的:探讨分析主动脉夹层患者的心理社会因素的特点。方法:应用临床心理测定工具分别评价62例主动脉夹层患者及62例正常对照人群的生活事件、人格特征、应付方式、社会支持、患病后的心身健康状况及其影响因素。结果:病例组患病前所经历的生活事件频数及紧张值,消极应付方式评分,抑郁及焦虑分值均显著高于对照组(P<0.05)。病例组积极应付评分、社会支持总分低于对照组(P<0.05)。生活事件负性紧张、焦虑和抑郁为主动脉夹层的危险因素,而积极应付方式和社会支持为疾病的保护因子。结论:本研究发现主动脉夹层患者具有特殊的心理社会特征和心身健康状况,除了常规治疗同时,应采取积极的社会心理干预措施。