慢性骨髓增殖性疾病并发肺动脉高压的临床特点和预后分析

目的:探讨慢性骨髓增殖性疾病(CMPD)并发肺动脉高压(PAH)的临床特点及PAH对于CMPD临床预后的影响,进一步丰富CMPD的评估体系.方法:选取CMPD并发PAH病例11例,选取同期CMPD肺动脉压正常病例27例作为对照.结果:与肺动脉压正常的CMPD病例相比,CMPD并发PAH平均年龄及血小板计数明显升高,且有...     

常见骨髓增殖性疾病骨髓病理学特征

2008年WHO造血与淋巴组织肿瘤分类（简称2008年WHO分类）将慢性骨髓增殖性疾病（chronic myeloproliferative disease,CMPD）称为骨髓增殖性肿瘤（myeloproliferative neoplasm,MPN）,并在2001年WHo分类的CMPD基础上增加了肥大细胞增生症（mastocytosis,MC）,共8种亚型。慢性中性粒细胞白血病（chronic neutrophilic leukemia,CNL）、慢性嗜酸粒细胞白血病,非特指型（CEL,NOS）、MC和MPN,无法分类（MPN,U）均相对少见,故本文仅对其中常见的真性红细胞增多症（polycythemia vera,PV）、原发性骨髓纤维化（idiopathic myelfibrosis,IMF）和原发性血小板增多症（essential thrombocythemia,ET）的骨髓活检病理诊断与鉴别做一介绍。     

胶原性疾病合并肺动脉高压

肺动脉高压的病因极其复杂,本文重点讨论胶原性疾病合并肺动脉高压的临床特征及实验室结果,报道三例系统性红斑狼疮、类风湿性关节炎、混合性结缔组织病等并发肺动脉高压患者     

血细胞去除术在骨髓增殖性疾病中的治疗作用

慢性骨髓增殖性疾病(CMPD)是一组克隆性造血干细胞病，共同特点为骨髓中髓系细胞(粒细胞、单核细胞、红细胞、巨核细胞)程度不等的增殖，成熟分化基本正常，以至外周血粒细胞、红细胞和(或)血小板数增多，如真性红细胞增多症、特发性血小板增多症及慢性粒细胞白血病等。由于血细胞过度增殖，患者大多出现血液淤滞、黏稠度增加，     

高龄老年慢性骨髓增殖性疾病转化的急性粒细胞白血病1例

1例慢性增殖性疾病的高龄老年患者（82岁）,予羟基脲治疗,并按血小板、白细胞数调整剂量;此后转化为急性粒细胞白血病,增加羟基脲剂量的同时出现粒细胞缺乏及感染,加之患者高龄、基础疾病多,给治疗带来极大难度。经血液科、呼吸内科会诊综合调整,加强支持治疗,现患者一般情况尚可,病情相对稳定,但总体预后不佳。如何针对高龄老年血液病完善治疗,是非血液专业的老年科医师亟待加强的问题。     

骨髓增生异常/骨髓增殖性疾病1例并文献复习

目的：了解骨髓增生异常/骨髓增殖性疾病（MDS/MPD）的临床特征及治疗。方法：分析1例典型MDS/MPD病例的诊疗经过,并进行相关文献复习。结果：患者诊断MDS/MPD明确后进行积极治疗,病情基本稳定。结论：MDS/MPD是一种少见的疾病,具有骨髓增生异常（MD）和慢性骨髓增殖性疾病（MPD）双重特点,目前尚无特异性疗法。     

JAK2V617F突变分型和相对定量研究——附123例慢性骨髓增殖性疾病检测结果分析

目的 研究JAK2V617F突变在慢性骨髓增殖性疾病(CMPD)中的发生率和突变类型,并对突变转录本水平进行定量分析.方法 对2003年1月至2008年5月苏州大学附属第一医院就诊的CMPD患者采用突变特异性扩增系统(ARMS)PCR方法检测JAK2V617F的发生率及其突变类型;采用毛细管电泳方法定量分析JAK2V617F突变转录本水平.结果 123例CMPD患者共检出90例JAK2V617F阳性,其中35例真性红细胞增多症(PV)患者中JAK2V617F阳性率100%,85例原发性血小板增多症(ET)患者中JAK2V617F阳性率62.4%,低于PV患者,差异有统计学意义(P<0.05);3例慢性骨髓纤维化(IMF)患者中JAK2V617F阳性率66.7%.90例JAK2V617F突变患者中共检出纯合突变35例,其中PV患者17例(17/35),占48.6%,ET患者17例(17/85),占20.0%,低于PV患者,差异有统计学意义(P<0.05),IMF患者1例;毛细管电泳定量分析显示,纯合型突变患者JAK2V617F突变转录本水平较杂合型突变患者低,差异有统计学意义(P<0.05);杂合型PV患者JAK2V617F突变转录本水平高于杂和型ET患者,差异有统计学意义(P<0.05).93例患者进行了染色体检查,6例有核型异常,但未发现特异性染色体改变.结论 ARMS-PCR可作为JAK2V617F突变较准确的检测方法,结合毛细管电泳可用于此突变的定量分析以及临床CMPD的诊断.     

重视骨髓增殖性疾病的诊治与研究

1951年美国著名的血液病学家Dameshek提出：真性红细胞增多症（polycythemia vera，PV）、慢性髓细胞白血病（chronic myelocytic leukemia，CML）、原发性血小板增多症（essential thrombaythemia，ET）和原发性骨髓纤维化（primary myelofibrosis，PMF）这4种慢性血液病可统称为“骨髓增殖性疾病（myeloproliferative disease，MPD）”，因为它们有类似的临床表现和病理基础。此后，随着细胞遗传学技术的进步，确定MPD是一组多能造血干细胞的克隆性疾病。MPD在欧美国家中并不少见，总的发生率为6～10／（10万人口·年。好发于50岁以上的中、老年人，男性多见。在中国尚无可靠的流行病学调查。     

西地那非治疗老年慢性心力衰竭并发肺动脉高压的效果

目的 观察西地那非治疗老年慢性心力衰竭（CHF）并发肺动脉高压（PH）的效果。方法 将86例老年CHF并PH患者随机分为两组:对照组和试药组（每组43例）。对照组采用常规治疗方法,试药组在此基础上使用西地那非治疗,比较两组患者的治疗效果、左心功能、肺动脉收缩压（PASP)、超敏C反应蛋白（hs-CRP）及氨基末端脑钠尿肽前体（NT-pro-BNP）水平。结果 试药组治疗后左室射血分数（LVEF)（42±11）%、左室短轴缩短率（LVFS）（26±7）%和6分钟步行距离（6MWT）（423±52）m,明显高于对照组的（39±8）%、（23±6）%和（361±32）m,差异均有统计学意义（均P<0.05）;试药组治疗后PASP（36±9）mmHg、hs-CRP（9±4）mg/L及NT-pro-BNP（535±49）ng/L均明显低于对照组的（40±8）mmHg、（13±4）mg/L和（796±97）ng/L,两组间差异显著（均P<0.05）。结论 西地那非治疗老年CHF并PH效果显著优于常规治疗。     

慢性骨髓增殖性疾病131例JAK2V617F突变的定量分析及临床意义

目的 研究JAK2V617F突变在慢性骨髓增殖性疾病(CMPD)中的发生率、突变类型、转录本水平及其临床意义.方法 采用突变特异性扩增系统(ARMS)-PCR方法 检测JAK2V617F的发生率及其突变类型;毛细管电泳方法 定量分析JAK2V617突变转录本水平.结果 纯合型JAK2V617F突变特发性血小板增多症(ET)患者和杂合型JAK2v617F突变ET患者其发病年龄均较野生型患者为高(P<0.05);CMPD患者发病年龄与JAK2V617F突变的发生问存在相关性(P<0.05),≥60岁患者的JAK2V617F突变转录本水平高于<60岁患者(P<0.05);JAK2V617F突变型CMPD患者中真性红细胞增多症(PV)和ET患者白细胞和血红蛋白水平均高于野生型患者(P<0.05),ET患者中纯合型突变者白细胞水平高于杂合型突变者(P<0.05);JAK2V617F突变在PV中的发生率和JAK2V617F纯合型突变在PV中的发生率均高于ET患者(P<0.05);JAK2V617F转录本水平均值在PV、ET和慢性特发性骨髓纤维化患者中无差别.结论 ARMS-PCR结合毛细管电泳定性和定量分析JAK2V617F突变可用于CMPD的诊断和预后判定.     

Unexplained pulmonary hypertension in chronic myeloproliferative disorders

AIM: To investigate the potential association between the chronic myeloid disorders (CMDs), including the chronic myeloproliferative disorders, and pulmonary hypertension (PH). METHODS: Retrospective chart review of patients who had received diagnoses of both CMD and PH. Patients with a known cause of PH were excluded. The diagnosis of a CMD was based on established criteria. The diagnosis of PH was based on echocardiographic data or right heart catheterization data. RESULTS: Twenty-six patients satisfied the criteria for both a CMD and PH. Twelve patients had myeloid metaplasia with myelofibrosis (MMM), 5 patients had essential thrombocythemia (ET), 6 patients had polycythemia vera, 2 patients had a myelodysplastic syndrome, and 1 patient had chronic myeloid leukemia. Twenty-two patients (92%) received treatment for their CMDs, which included therapy with hydroxyurea (18 patients), anagrelide (7 patients), and busulfan (3 patients). PH was diagnosed a median of 8 years after recognition of the CMD (range, 0 to 26 years). The median right ventricular systolic pressure (RVsys) was 71 mm Hg (range, 32 to 105 mm Hg). RVsys correlated with the platelet count in patients with MMM (r = 0.30) and ET (r = 0.6) and with the hemoglobin levels in patients with PV (r = 0.77). Treatment of CMD did not seem to affect the severity of the pulmonary artery pressures as measured by serial echocardiography. With a median survival time of 18 months after the diagnosis of PH, the cause of death in the majority of the patients was cardiopulmonary. CONCLUSIONS: The current study suggests a higher than expected incidence of PH in patients with MMM, PV, and ET. Prognosis in such a setting is poor and may not be influenced by aggressive treatment of the underlying hematologic disorder.     

维族老年慢性阻塞性肺疾病患者合并肺动脉高压的临床研究

目的 分析维族老年慢性阻塞性肺疾病（COPD）患者合并肺动脉高压（PH）的患病情况和临床特征。方法 2014年2月至2015年5月在新疆莎车县人民医院综合内科确诊的稳定期维族老年COPD患者218例,根据心脏彩色多普勒超声分为非PH组（96例）、可疑PH组（68例）和确定PH组（54例）。比较PH组与非PH组的年龄、性别、体质量指数、疾病病史、肺功能检查、血气分析、血红蛋白（HGB）和脑钠肽前体（pro-BNP）等的差异。结果 PH组比非PH组的慢性咳嗽咯痰病史更长久（P＜0.01）,合并糖尿病的概率更高（P＜0.05）,HGB和pro-BNP的水平也偏高（均P＜0.01）。PH组第一秒用力呼气容积占预计值的百分比（FEV1%pred）、一氧化碳弥散量占预计值的百分比（DLCO% pred）和动脉血氧分压（PaO2）的测定值也均比非PH组高（P＜0.01）,其他肺功能指标差异无统计学意义（P＞0.05）。结论 如能及早重视患者上述临床特征,并给予规范治疗,或可延缓维族老年COPD患者合并PH的进程并改善预后。     

慢性肺心病合并冠心病41例临床分析

目的 探讨慢性肺心病（肺心病）合并冠心病的临床特点.方法 对41例肺心病合并冠心病患者（肺冠合并组）和30例单纯肺心病（单纯肺心组）进行临床比较、统计、分析临床特点及诊断方法.结果 在易患因素上,两组在长期吸烟、高血压、高脂血症、肥胖等有显著性差异（P＜0.05或P＜0.01）.在临床表现方面,其左心室肥大、双心室增大、合并左束枝传导阻滞,持续ST-T改变等均有显著性差异（P＜0.01）.结论 慢性肺心病患者如伴有高血压、糖尿病、肥胖、左心室肥大、持续ST-T改变,心梗/左心衰时,在排除少数相关疾病后即可临床诊断为肺心病合并冠心病.     

慢性心力衰竭伴低钠血症患者102例临床分析

目的 探讨3%氯化钠治疗慢性心力衰竭伴低钠血症患者的临床疗效.方法 对102例慢性心力衰竭(纽约心功能分级Ⅲ-Ⅳ级)伴低钠血症患者,给予限水、扩张血管、强心、利尿、血管紧张素转换酶抑制剂/血管紧张素II受体拮抗剂等常规治疗外,同时给予3%氯化钠.比较治疗前后患者的心功能分级、治疗效果及血钠水平.结果 102例患者治疗后,心功能恢复至Ⅰ级39例,心功能恢复至Ⅱ级47例,心功能Ⅲ级11例,心功能Ⅳ级5例.与治疗前心功能Ⅲ级45例,Ⅳ级者57例比较,差异有统计学意义(χ2=150.26,P＜0.05).治疗后显效58例,37例有效,7例无效,总有效率93.1%.治疗后心功能Ⅲ级、Ⅳ级患者的血钠分别(139.4±22.6) mmol/L、(138.2±23.3)mmol/L,与治疗前心功能Ⅲ级、Ⅳ级患者的血钠(122.9±20.5)mmol/L、(120.5±26.8)mmol/L比较,差异有统计学意义(t=2.02、2.04,均P＜0.05).结论 合理利尿、适当补充钠盐有助于减少慢性心力衰竭伴低钠血症患者不良事件的发生,改善患者预后.

Abstract：

Objective To summarize the clinical experience of 3% sodium chloride in treatment of chronic heart failure with hyponatremia. Methods The 102 patients suffering from chronic heart failure with hyponatremia were treated with water limit, vasodilator, cardiotonic, diuretics, angiotensin-converting enzyme inhibitor, angiotensin Ⅱ receptor antagonist and 3% sodium chloride. Cardiac functional capacity classification (NYHA), treatment effect and hyponatremia level were compared between pre- and post- treatment. Results After treatment, heart function was statistically different as compared with that of pre-treatment (χ2=150.26, P＜0.05). The 58 cases showed distinct effect, 37 cases moderate effect, 7 cases no effect, and the percentage of total effect was 93.1%. The hyponatremia of patients with different level of heart function were (139.4±22.6) mmol/L, (138.2±23.3) mmol/L, the difference had a statistics significance compared with pre-treatment hyponatremia which were (122.9±20.5) mmol/L, (120.5±26.8) mmol/L (t=2.02, 2.04, P＜0.05). Conclusions Proper diuresis and sodium salt supplement could help to reduce the malignant risk of the patients suffering from chronic heart failure with hyponatremia and to improve the prognosis.     

Two cases of unclassified chronic myeloproliferative disorders]

Two cases of unclassified chronic myeloproliferative disorders (UCMPD), diagnosed by hematological, cytogenetic and DNA analyses, are described. Case 1: a 63 year old female was admitted because of leukocytosis (96,800/microliters) and splenomegaly. Hematological examinations revealed an increase of the granulocytes in the peripheral blood and bone marrow. The neutrophil alkaline phosphatase (NAP) score was 121. The patient developed blast crisis after 12 months of the chronic phase. Case 2: a 48 year old male was presented with fever and leukocytosis (20,000/microliters). Hematological examinations revealed an increase of granulocytes in the peripheral blood and bone marrow. The NAP score was 33. Maturation-arrest in granulocytic series and morphological abnormalities of marrow cells were not observed in the two cases. Cytogenetic analysis of bone marrow cells disclosed 46, XX, i (17 q) in case 1 and 47, XY, +8 in case 2. Southern blot analysis using 3' bcr probe and TransProbe-1 showed no bcr rearrangement. These cases are thought to be valuable in order to clarify the relationship between UCMPD and CMPD such as Ph1 negative chronic myelocytic leukemia and myelodysplastic syndromes.     

慢性阻塞性肺病并发自发性气胸56例分析

目的 分析慢性阻塞性肺病并发自发性气胸的临床特点，提高临床诊治水平。方法对2001年～2008年确诊为慢性阻塞性肺病合并自发性气胸56例患者的临床资料进行回顾性分析。结果误诊率达32．1％。结论慢性阻塞性肺病并发自发性气胸临床表现不典型，误诊率较高，病情发展快，常威胁病人生命，能及时诊断和治疗，可降低死亡率。