三氧化二砷联合化疗治疗急性早幼粒细胞白血病的疗效观察

目的:观察三氧化二砷(As2O3)联合化疗治疗急性早幼粒细胞白血病(APL)的疗效.方法:46例初治APL患者均采用As2O3诱导治疗,完全缓解(CR)后用DA或MA方案巩固1个疗程,以后As2O3与化疗交替治疗,每3月1次,连续治疗3年.再继以甲氧蝶呤片与巯嘌呤片维持治疗1年.结果:46例患者有38例(82.6％)达到CR,34例坚持治疗者持续缓解,缓解时间17～119个月,中位缓解时间67个月;4例CR2患者缓解时间43～ 71个月,中位缓解时间54个月.38例患者仍处于CR,生存时间131～ 18个月,中位生存时间65个月.结论:APL患者CR后,采取每年4个周期的巩固和维持方案序贯治疗,减少了化疗及As2O3的疗程,避免了长期连续用药所致的耐药,减少了药物的副作用,复发率很低.     

Clinico-Pathological Spectrum of Primary Plasma Cell Leukemia Diagnosed at a Tertiary Care Centre in South India Over 5?Year Period

Plasma cell leukemia (PCL) represents a rare and aggressive form of plasma cell dyscrasia which can be primary (pPCL) or secondary (sPCL). It is diagnosed based on absolute plasma cell count of more than 2.0 × 10⁹/l or a relative proportion of greater than 20% of the peripheral blood leukocyte count. Although pPCL and sPCL share several clinical features, important differences exist. Patients with pPCL are younger; often have extra osseous organ involvement (liver, spleen and other extramedullary sites), increased frequency of renal failure, fast declining performance status and rapid progression to the terminal stage. Patients with sPCL have advanced bone disease. Presented in this article is India data of a short series of five cases of PCL diagnosed at a tertiary care centre from south India over last 5 years. All cases were de novo and had varied spectrum of presentation and so were not suspected to be plasma cell dyscrasia clinically. Detailed hemato-pathological evaluation clinched the diagnosis in all the cases.     

急性白血病患者血清促红细胞生成素变化及其与化疗的关系

目的 分析急性白血病患者血清促红细胞生成素(Epo)水平及其与血红蛋白(Hb)的关系,以及化疗对其的影响.方法 应用ELISA法检测急性白血病患者血清Epo水平,并观察化疗后Epo变化.结果 32例急性白血病患者化疗前Epo水平(49.58±14.87mIU/mL)高于健康对照者(11.96±4.78mIU/mL)(P＜0.01);Epo水平与Hb水平存在负相关(r =-0.541,P＜0.01);化疗2疗程和4疗程后,Epo水平分别为52.12±15.16mIU/mL和63.15±16.42mIU/mL,与化疗前比较,差异有统计学意义(F =4.547,P＜0.01);化疗2疗程和4疗程后一周,Hb分别为62.0±12.3g/L和56.8±11.1g/L,与化疗前Hb(78.0±14.9g/L)比较,差异有统计学意义(F=4.007,P＜0.01).结论 急性白血病患者Epo合成机制完整,虽然贫血,但EPO水平是增高的,化疗抑制期内随着贫血加重,Epo水平升高.     

急性白血病患儿化疗期间骨髓抑制合并感染时降钙素原意义

目的：探讨降钙素原在急性白血病患儿化疗期间骨髓抑制期合并感染时的意义。方法采用免疫荧光层析法测定我科急性白血病患儿化疗后骨髓抑制时合并感染时降钙素原值共102例,同时选取化疗后未发生感染患儿50例作为对照,采用回顾性研究方法将102例感染患儿按照感染类型分为细菌感染组(50例)与非细菌感染组(52例),按照易感程度分为低危感染组(27例)与高危感染组(75例)分别进行对照分析。结果儿童急性白血病骨髓抑制合并细菌感染时降钙素原明显高于非细菌感染组,而非细菌感染组明显高于对照组,感染危险程度对于降钙素原无影响。结论降钙素原可以作为儿童急性白血病化疗期间合并感染时判断细菌感染与非细菌感染的敏感指标。     

CD26 a cancer stem cell marker and therapeutic target

Cancer stem cells (CSCs) comprise a tumor subpopulation responsible for tumor maintenance, resistance to chemotherapy, recurrence and metastasis. The identification of this cell group is very important, but there is still no consensus on its characterization. Several CSC markers have been described, like CD133, CD24, CD44 and ALDH1, but more research to identify new markers to facilitate the identification of CSC in a heterogeneous tumoral mass is required. Thus, this article describes the CD26 expression as a CSC marker and the role that it plays in different types of cancer. CD26 expression correlates with some characteristics of CSCs, like the formation of spheres in vitro, formation of new tumors, and resistance to chemotherapy. CD26 is therefore suggested as an auxiliary marker for CSC in different types of cancer, and as a potential therapeutic target.     

372例骨髓细胞形态学诊断结果分析

目的研究骨髓细胞形态学结果分布特点及在血液病病因诊断中的作用,了解该地区血液病疾病谱特征。方法2012年3月至2014年4月该院372例血液病患者的骨髓穿刺标本进行瑞姬氏染色和化学染色,显微镜观察细胞形态和化学染色结果,并结合相关临床资料得出形态学报告。结果372例患者确诊为血液系统疾病368例,以白血病(95/372)、增生性贫血(36/372)和缺铁性贫血(26/372)为3种主要类型。82例贫血患者类型分析,以增生性贫血(36/82)、缺铁性贫血(26/82)和巨幼细胞性贫血(11/82)为主。结论骨髓细胞形态学检查是血液系统疾病病因诊断的重要手段之一,对疾病谱的分析有助于指导临床诊治。     

179例血液病患者EBV和HCMV核酸检测阳性率与患者年龄的关系

目的了解再生障碍性贫血、急性淋巴细胞白血病及髓系白血病患者EB病毒(EBV)和人巨细胞病毒(HCMV)核酸检测阳性率与其年龄的关系。方法收集中国人民解放军海军总医院2012年1月至2013年12月骨髓穿刺后临床确诊为再生障碍性贫血、急性淋巴细胞白血病及髓系白血病患者为研究对象,采用达安基因核酸检测试剂盒对EBV和HCMV核酸进行检测,分析EBV和HCMV感染阳性率与患者年龄的关系。结果 EBV和HCMV在3种血液病中核酸检测阳性率不同,HCMV核酸检测阳性检出率(15.8%)低于EBV(43.7%)。再生障碍性贫血和髓系白血病患者中,不同年龄患者EBV核酸检测阳性率比较差异有统计学意义(P0.01)。结论对于儿童再生障碍性贫血病和髓系白血病患者应更加重视EBV和HCMV核酸的监测。     

慢性淋巴细胞白血病CD5漏检临床案例分析及对策

目的分析不同荧光素标记抗体对慢性淋巴细胞白血病(CLL)B细胞表面CD5检测结果差异及其对临床诊断的影响。方法采用流式细胞仪检测3例CD5表达强度不同的慢性淋巴细胞白血病(CLL)患者的免疫表型,采用SYSMEX XE2100全自动血液分析仪检测血常规;以1例急性B淋巴细胞白血病(B-ALL)患者作为阴性对照。结果采用异硫氰酸荧光素(FITC)标记抗体检测4例患者骨髓异常B淋巴细胞CD5表达,CD5阳性细胞未见独立成群,阳性细胞比例分别为47.1%、17.7%、6.7%和7.9%;以≥20%为标准,仅病例1阳性。以PE-Cy7荧光素标记抗体重新检测,病例1和2的CD5阳性细胞独立成群,病例3 CD5表达呈连续表达模式,阳性细胞比例分别上升至91.1%、70.3%和38.2%。而对照病例4(B-ALL患者)2次检测CD5比例均为阴性。病例1、2、3符合典型CLL免疫表型。结论 CLL患者B细胞表面常异常表达CD5,但其表达强度显著低于T细胞表面CD5表达。采用弱荧光素标记抗体检测B细胞表面CD5表达,会出现漏检进而影响临床诊断。对弱表达抗原选择合适的荧光素标记,并进行验证和比对确定其准确性,是正确提供疾病免疫表型的重要保障。     

Focal myocardial fibrosis assessed by late gadolinium enhancement cardiovascular magnetic resonance in children and adolescents with dilated cardiomyopathy

Background

Different patterns of late gadolinium enhancement (LGE) including mid-wall fibrosis using cardiovascular magnetic resonance (CMR) have been reported in adult patients presenting with non-ischemic dilated cardiomyopathy (DCM). In these studies, LGE was associated with pronounced LV remodelling and predicted adverse cardiac outcomes. Accordingly, the purpose of our study was to determine the presence and patterns of LGE in children and adolescents with DCM.

Methods

Patients <18 years of age presenting with severe congestive heart failure who were admitted for evaluation of heart transplantation at our centre underwent CMR examination which consisted of ventricular functional analysis and assessment of LGE for detection of myocardial fibrosis. Ischemic DCM was excluded by coronary angiography, and right ventricular endomyocardial biopsies ruled out acute myocarditis.

Results

Thirty-one patients (mean age 2.1 ± 4.2 years) with severe LV dilatation (mean indexed LVEDV 136 ± 48 ml/m²) and LV dysfunction (mean LV-EF 23 ± 8%) were examined. LGE was detected in 5 of the 31 patients (16%) appearing in various patterns characterized as mid-wall (n = 1), focal patchy (n = 1), RV insertion site (n = 1) and transmural (n = 2). Based on histopathological analysis, 4 of the 5 LGE positive patients had lymphocytic myocarditis, whereas one patient was diagnosed with idiopathic DCM.

Conclusions

In children and adolescents with DCM, focal histologically proven myocardial fibrosis is rarely detected by LGE CMR despite marked LV dilatation and severely depressed LV function. LGE occurred in various patterns and mostly in patients with inflammatory cardiomyopathy. It remains unclear whether myocardial fibrosis in childhood DCM reflects different endogenous repair mechanisms that enable favourable reverse remodelling. Larger trials are needed to assess the prognostic implications of LGE in childhood DCM.     

Report on Third International Intensive Aftercare Conference in Norrköping,Sweden

The third international conference on intensive care unit (ICU) diaries and intensive aftercare took place in Norrköping, Sweden, on 28 November 2013. The conference was organized by Carl Bäckman and colleagues, Vrinnevi Hospital and NOFI, and represented by Sissell Storli. More than 100 clinicians from across Europe and the USA attended the conference.