骨髓增生异常综合征35例分析

本文作者通过对骨髓增生异常综合征35例的血象、骨髓象、细胞化学染色等进行了分析,并结合临床表现,讨论了分型、诊断、鉴别诊断、转归等问题.     

骨髓增生异常综合征异常克隆在病态和非病态造血细胞中的分布

1资料与方法1.1病例资料收集我院诊治的5例骨髓增生异常综合征(MDS)患者,男3例、女2例,中位年龄66(53～91)岁。按WHO标准分型:1例为含5q-的复杂核型异常,诊断为难治性血细胞减少伴多系发育异常(RCMD),骨髓红系和粒系均有病态造血,巨     

骨髓增生异常综合征患者肺功能的临床研究

目的探讨骨髓增生异常综合征（myelodysplastic syndrome,MDS）患者肺功能情况,分析MDS患者行异体造血干细胞移植术后肺功能的变化。方法选取海军总医院2013年1月—2017年10月收治的50例MDS患者为观察组,同期健康体检者50例为对照组,回顾性比较2组患者用力肺活量（forced vital capacity,FVC）、第1秒用力呼气容积（forced expiratory volume in one second,FEV1）、肺活量（vital capacity,VC）、残气容积（residual volume,RV）、最大呼气流量（peak expiratory flow,PEF）、最大呼气中期流量（maximal mid expiratory flow,MMEF）、肺一氧化碳弥散量（diffusing capacity of the lung for carbon monoxide,DLCO）占预计值百分比等各项肺功能指标的差异。15例MDS患者行异体造血干细胞移植治疗,比较移植前与移植后6～12月内肺功能的变化。结果观察组患者FVC、FEV1、VC、PEF、MMEF、DLCO占预计值百分比降低,与对照组相比差异有统计学意义（P<0.05）,RV占预计值百分比增高,但差异无统计学意义。肺功能指标特别是通气功能指标与患者患病时程、肺部感染次数和联合化疗呈负相关。MDS患者行异体造血干细胞移植术后FVC、FEV1、VC、PEF、MMEF、DLCO占预计值百分比较移植前降低（P<0.01）。结论MDS患者肺通气功能、小气道功能和弥散功能均受损害,肺功能指标与患者患病时程、肺部感染次数和联合化疗呈负相关。患者行异体造血干细胞移植术后通气功能、小气道功能和弥散功能受损加重。     

41例骨髓增生异常综合征血象骨髓象分析

骨髓增生异常综合征(myelodysplastic syndrome,MDS)是一组克隆性造血干细胞疾病,是白血病前期骨髓造血功能异常的阶段。MDS判定除了要以骨髓象改变为主要标准之外,应密切参考血象变化及临床表现。本文就1989-2002年收治的41例MDS患者的血象与骨髓象作分析如下。     

骨髓增生异常综合征的骨髓MRI研究

目的研究骨髓增生异常综合征(myelodysplastic syndromes,MDS)和急性髓性白血病(acute myeloid leukemia, AML)患者股骨骨髓MRI表现,评价MRI在MDS诊断、病情评估及预后预测方面的临床应用价值.方法搜集经临床表现及细胞形态学综合分析诊断为MDS 16例,AML 14例,行骨盆及股骨中上段骨髓MRI,采用SE及短时反转恢复(STIR)序列行正冠状面及双侧斜冠状面扫描,选取30例排除血液系统疾病的相对应年龄志愿者作为对照组.搜集MDS及AML患者同期骨髓象及血常规结果和MRI对比分析.结果患者骨髓MRI表现按浸润程度和范围分为3级,MDS患者4例属Ⅰ级,6例属Ⅱ级,6例属Ⅲ级;AML患者8例属Ⅱ级,6例属Ⅲ级,无Ⅰ级病例.MDS与AML患者MRI分级总体的分布差异无显著性意义(t=1.388,P>0.05).比较骨髓率,MDS组与AML组同对照组间差异均有显著性意义(t值分别为3.032和2.9051,P值均<0.05);MDS组与AML组骨髓率分级分布差异有显著性意义(t=8.988,P<0.05).结合MRI表现及骨髓率评分的MRI分级与临床诊断指标有非常显著一致性(χ2=6.218,P<0.01).结论单纯以MRI表现为依据的分级对于鉴别MDS与AML价值有限,MRI表现及骨髓率评分相结合的分级标准综合临床资料分析对MDS的诊断、病情评估及预后预测有重要的临床应用价值.     

小儿骨髓增生异常综合征16例临床分析

小儿骨髓增生异常综合征１６例临床分析武警陕西总队医院儿科焦丽，刘志刚，郭新莉（西安７１００５４）自１９８２年ＦＡＢ协作组提出骨髓增生异常综合征（ＭＤＳ）的诊断及分型以来［１］，已引起国内外血液学家的重视．国内外学者对成人ＭＤＳ报道甚多，而小儿ＭＤＳ报...     

婴儿骨髓增生异常综合征1例报告

患儿 ,女 ,5个月。 2 0d前无明显诱因发热 ,体温 39℃ ,无咳嗽、腹泻 ,当地医院查血常规WBC 9.0× 1 0 9/L ,Hb 1 1 4g/L ,异型淋巴细胞 1 4 % ;腹部B超提示肝、脾肿大 ;骨髓细胞学检查提示增生骨髓象 ,给予抗感染、抗病毒治疗。入院查体 :意识清楚 ,体温 38.1℃ ,枕秃 ,全身皮肤黏膜无黄染 ,腋下及背部可见红色皮疹 ,枕部及颈部可触及数枚直径 0 .5cm肿大淋巴结 ,咽无充血 ,两肺呼吸音粗糙 ,闻及粗湿音 ,心率 1 1 0次 /min律齐 ,未闻及病理性杂音 ,腹软 ,肝脏肋下 4cm ,质软 ,脾脏肋下 3cm ,右侧未超过中线 ,质软 ,移动性浊音阴性 ,肠…     

同胞脐血移植治疗儿童骨髓增生异常综合征国内首例成功报告

目的探讨脐血移植对儿童骨髓增生异常综合征的治疗效果。方法 1例12岁患儿行人白细胞抗原相合的同胞脐血移植,预处理采用白消安/环磷酰胺方案:白消安1mg/kg,每6 h一次,用2 d,共8次;环磷酰胺50mg/(kg·d),用4 d;抗胸腺球蛋白100 mg/d,用4 d。输入脐血有核细胞2.57×1 07/kg,CD34+细胞1.18×105,kg。移植物抗宿主疾病的预防采用环胞菌素A+骁悉+甲泼尼龙。移植后应用粒细胞集落刺激因子、白细胞介素-11及促红细胞生成素以加速造血重建。结果 +2l天粒系植入,+48天血小板植入,+28天患者骨髓DNA指纹图示完全嵌合状态。随访11个月,患者各项检查正常,未发生急、慢性移植物抗宿主疾病。结论本例为国内大陆首例成功脐血移植治疗骨髓增生异常综合征,为今后儿童骨髓增生异常综合征的治疗提供了一种新的方法。     

骨髓增生异常综合征的MRI研究进展

骨髓增生异常综合征（MDS）是一组以无效造血为特征的骨髓疾病,特点是向急性髓系白血病（AML）转化风险高。MDS一旦转化为AML,病人将面临治疗难度增加、预后变差等诸多问题,故了解MDS向AML转化的征象、预测MDS向AML的转化具有重要意义。MRI能够从整体上显示骨髓结构并具有无创性,其有助于MDS的诊断、病情评估及预测MDS转化为AML的风险。就MRI在MDS中的研究进展进行综述。     

骨髓增生异常综合征和巨幼细胞性贫血患者血清胆红素、乳酸脱氢酶的变化和临床意义

目的：探讨血清胆红素,乳酸脱氢酶在骨髓增生异常综合征,巨幼细胞性贫血患者中的变化和的临床意义。方法：采用国际标准酶法空腹测定血清总胆红素、间接胆红素及乳酸脱氢酶,并对临床及实验资料作回顾性分析。结果：巨幼细胞性盆血组与骨髓增生异常综合征组相比较血清乳酸脱氢酶及胆红素水平明显增高,差异有统计学意义,P〈0.05。结论：血清乳酸脱氢酶、血清胆红素水平可作为骨髓增生异常综合征与巨幼细胞性盆血鉴别诊断的血清学指标。     

Treatment of testicular tumors: analysis of treatment results of chemotherapy

Between 1968 and 1982 altogether 72 patients with malignant testicular tumors were treated at the Department of Radiotherapy of the Turku University Central Hospital. 49 patients had seminoma and 21 had non-seminoma, one had histiocytoma and one had sarcoma. The mean age of the patients was 39 years, and the mean age of those 25 who received chemotherapy was 25 years. Primary treatment was surgical for all patients; four patients had no further treatment, while the remaining patients received radiotherapy and/or chemotherapy. 25 patients received chemotherapy. Seven of them were treated with one to seven courses of "triple drug therapy" (dactinomycin-chlorambucil-methotrexate). These patients died within two years from the time of dissemination. Ten patients were given different combinations or monotherapies and all except two patients, died within two years. CBV combination therapy was instituted in the 1970's and during the study period eight patients were treated with this combination. Six complete remissions were achieved. Two patients died of progressive disease. Chemotherapy was given for two to 30 months. The mean follow-up time for these patients was three years (32 to 42 months). Chemotherapy caused significant morbidity, but no long-lasting irreversible toxic side effects were detected. The results of the present study are promising and correspond to those reported elsewhere.     

大剂量甲氨喋呤治疗儿童急性淋巴细胞白血病的不良反应观察及临床对策

目的研究大剂量甲氨喋呤（HD-MTX,5g／m^2）加四氢叶酸钙（CF）解救方案治疗儿童急性淋巴细胞白血病（ALL）的不良反应。方法61例（173例次）的HD—MTX加CF治疗,监测44hMTX血药浓度。观察HD—MTX化疗的各种副作用,并给予相应治疗。结果骨髓抑制、胃肠道反应、皮肤黏膜损害、感染、肝功能损害最为常见,大部分都能在给予相应处理后得到控制。结论HD—MTX（5g／m^2）加CF治疗儿童白血病时,不良反应较为常见,但大多能及时控制,在有MTX血药浓度监测的条件下值得临床推广。     

异基因造血干细胞移植治疗儿童血液病

目的造血干细胞移植可以根治白血病、再生障碍性贫血、血红蛋白病及先天性免疫缺陷等疾病,通过临床观察,分析研究异基因造血干细胞移植对儿童血液病的治疗效果。方法采用异基因造血干细胞移植治疗15例儿童血液病,其中非血缘脐血移植9例,同胞脐血移植3例,同胞外周血干细胞移植2例,同胞骨髓移植1例。采用白消安、环磷酰胺或环磷酰胺、全身照射为基础的预处理的方案。结果14例患者植入,1例于移植后15d死亡,未达植入标准。白细胞的植入时间各组间没有明显差异;脐血移植血小板的植入明显较骨髓或外周血延迟（P〈0．05）。主要并发症为巨细胞病毒感染和复发,11例患者生存;其中10例无病存活,占66．7％,存活最长时间为6年。Kaplan-Meier生存曲线提示：1年生存率为76．6％,预计5年生存率为57．4％。结论脐血移植具有移植物抗宿主疾病轻、较容易控制、搜寻时间短等优点,对儿童患者具有广泛的应用前景。提出了儿童脐血移植的选择策略。     

Forensic issues at the extremes of weight: From frailty syndrome through frail obesity to morbid obesity

It is well recognised that there has been a striking increase in average body size (measured by the body mass index) in a number of countries over recent years, such that it has been referred to as an obesity epidemic. This has been associated with increased numbers of these cases in the forensic mortuary with the associated practical difficulties in body handling and the complexities of identifying multiple comorbidities. At the other extreme of body weight lies the frailty syndrome which manifests with unintentional weight loss, decreased muscle mass (sarcopaenia), reduced physical strength and activity, exhaustion and slow ambulation. Of note, morbid obesity and frailty syndrome involve the interaction of multiple environmental, genetic and neuroendocrine factors and are both characterised by systemic inflammatory responses and multifocal organ dysfunction. While cytokine upregulation could well be a secondary phenomenon, it appears that the extremes of weight are associated with significant metabolic disturbances and related diseases. It may be useful in forensic centres to identify decedents with either frailty syndrome or morbid obesity to enable better understanding of the true incidence of significant weight disturbance and its ramifications in medicolegal cases.     

25例AML患者白血病细胞端粒酶活性分析

目的探讨25例急性髓性白血病患者端粒酶活性的变化及意义.方法采用TRAP-ELISA-PAGE法测定端粒酶活性.结果AML患者端粒酶活性(吸光度A值为2.298±1.059)较正常对照(吸光度A值为0.387±0.598)显著增高.结论AML细胞端粒酶的活化可能在白血病的发生中起着重要的作用.     

指甲-髌骨综合征的临床及X线征象探讨(附一家族6例报告及文献复习)

目的提高对罕见病指甲-髌骨综合征的认识。方法结合文献复习,对6例该病的病理、临床及X线征象进行分析。结果本病的诊断要点为:(1)指甲发育异常及家族史;(2)病变为双侧性,多骨、多系统受累;(3)指甲、髂骨角、膝和肘部畸形为典型四联征表现。结论本病有典型的临床及X线表现,诊断并不困难,X线是诊断该病的主要检查手段。     

腭裂术后下颌关节紊乱的病因分析及治疗效果观察

目的探讨腭裂术后出现的颞下颌关节紊乱病的病因及治疗方法。方法根据颞下颌关节紊乱病的临床症状及病因,采取不同的治疗方法,如局部热敷、按摩咀嚼肌、口服止痛药、给予心理暗示和安慰等。结果经上述治疗后几乎所有患者的颞下颌关节区疼痛及张口受限均在短期内消失,仅有少数患者仍有颞下颌关节区弹响。结论腭裂修补术后并发颞下颌关节紊乱病极其常见,但经积极治疗后,多数患者的临床症状可在短时间内消失,预后较好。     

Treatment and results of sternoclavicular joint injuries

Sternoclavicular joint injuries are uncommon. The anatomy is not familiar to most orthopaedic surgeons, and there is a high risk of both intraoperative catastrophic and postsurgical complications when performing surgery in this region. Anterior SC joint instability should primarily be treated conservatively. The patients should be informed that there is a high risk of persistent instability with nonoperative or operative care, but that the persistent instability will be well tolerated and have little functional impact in the vast majority. Therefore, operative intervention for anterior SC joint instability is mainly cosmetic in nature. Patients with posterior SC joint dislocations require an expeditious diagnosis and treatment, due to the proximity of the displaced medial clavicle to the great vessels. An early closed reduction will usually be stable. Operative stabilization must, however, be considered if the closed reduction is unsuccessful or there is persistent SC instability. SC joint stabilization should be performed with a soft tissue reconstruction, repairing both the costoclavicular ligaments and the SC capsular envelope.     

度洛西汀与文拉法辛治疗躯体形式障碍的对照研究

目的 比较度洛西汀与文拉法辛治疗躯体化障碍的临床效果及安全性.方法 符合中国精神疾病分类与诊断标准第3版(CCMD-3)躯体形式障碍诊断标准患者90例,分为度洛西汀组与文拉法辛组各45例.度洛西汀组起始量为20mg/d,文拉法辛组起始量为50mg/d,于7~10d内分别加至60mg/d和200mg/d,治疗6周.采用汉密尔顿抑郁量表(HAMD)和临床疗效总评量表(CGI-SI)评定疗效,采用副反应量表(TESS)评定安全性.结果 度洛西汀组有效率为95.56%,文拉法辛组有效率为97.78%,两组无显著差异.两组HAMD、HAMA总分及因子治疗分在2、4、6周末与治疗前比较,均有非常显著差异(P<0.01).度洛西汀组的HAMD总分和睡眠障碍因子减分在治疗4周和6周末低于文拉法辛组(P<0.05,P<0.01),躯体化焦虑因子减分分别在治疗2周和6周末低于文拉法辛组(P<0.05).度洛西汀组的HAMA总分和躯体化焦虑因子减分在治疗2wk末大于文拉法辛组(P<0.05).两组不良反应按安全数据集分析,度洛西汀组比文拉法辛组多见困倦嗜睡、口干和便秘(P<0.05,P<0.01),其他不良反应无显著差异(P>0.05).结论 度洛西汀与文拉法辛治疗躯体化障碍的疗效相当,不良反应相似.