急性白血病患者生存率影响因素分析

报道46例急性白血病患者，其中生存21例，死亡25例。死亡患者生存期≥3a3例（死于中枢神经系统白血病1例、感染2例），〈3a22例（死于疾病复发20例，其中6例因自行停止化疗或延长化疗间歇期致疾病复发死亡）。提示加强中枢神经系统白血病防治、提高病情缓解后强化治疗阶段感染的防治及依从性，可提高部分急性白血病患者的生存率。     

191例AIDS死亡患者临床特征及死因分析

目的分析AIDS死亡患者的临床特征及主要死因。方法采用回顾性调查分析的方法,采集2009年8月—2014年4月上海市公共卫生临床中心所有AIDS住院患者中死亡病例的临床资料,采用Stata 10.0软件对数据进行统计分析。结果 AIDS死亡病例191例,中位年龄48岁,以男性(90.1%)为主。152例(79.6%)入院前尚未开始抗反转录病毒治疗。181例获得CD4+T淋巴细胞计数资料者,中位CD4+T淋巴细胞计数为21个/μl。入院首次实验室检查提示贫血118例(61.8%),低白蛋白血症147例(77.0%),低钾血症41例(21.5%),低钠血症89例(46.6%),肌酐升高16例(8.4%),尿素升高54例(28.3%)。合并肺部感染158例(82.7%),中枢神经系统(central nervous system,CNS)疾病54例(28.3%),消化道真菌感染56例(29.3%),AIDS相关肿瘤11例(5.8%)。并发感染性休克19例(9.9%),上消化道出血14例(7.3%),气胸及纵膈气肿9例(4.7%)。主要死因为肺部感染(53.9%)和CNS疾病(19.9%)。结论 AIDS死亡患者入院时一般情况差,合并症及并发症多见,主要死于肺部感染和CNS疾病。     

88 例老年非急性期脑卒中患者死因调查

目的探讨老年非急性期脑卒中患者主要死亡原因及其预防方法。方法对47例住院及41例家庭护理老年非急性期脑卒中死亡病例临床资料进行回顾性分析,将两组死亡原因及成活时间进行对比。结果死于肺炎共28例(31.8%),分别占两组第一位;住院组中死于原有疾病13例(27.66%),营养不良9例(19.15%),占第二、第三位;家庭组第二、三位死因分别是褥疮感染9例(21.95%)和意外7例(17.07%),两组死因构成差异有显著意义(P<0.05)。住院组存活时间平均(682±183)d,家庭组平均(456±107)d,(P<0.01)。结论肺炎是老年非急性期脑卒中患者死亡的主要原因,积极防治呼吸道感染、治疗原有疾病、加强护理、相关卫生知识宣教是提高患者生存质量、延长患者生命的关键。     

北京地坛医院免费抗病毒治疗HIV/AIDS死亡病人特征分析

目的了解北京地坛医院2005-2018年进行免费抗病毒治疗的艾滋病病毒(HIV)感染者中死亡病人的特征,为进一步降低死亡率提供依据。方法以北京地坛医院2005-2018年所有门诊免费抗病毒治疗的HIV感染者中的死亡病例为研究对象,进行回顾性分析,计算死亡率,分析死亡病例特征。结果共有7 171名HIV感染者在此期间进行免费抗病毒治疗,死亡88人,男80人,女8人,总死亡率0.33/100人年,死亡病人的平均年龄48.8岁。死于艾滋病(AIDS)相关疾病和非AIDS相关疾病病人的死亡率分别为0.21/100人年和0.07/100人年。63.6%(56/88)的病人死于AIDS相关疾病,死因前5位分别为:感染中毒性休克10例,结核病7例,淋巴瘤5例,除肺孢子菌肺炎外的肺部感染5例,隐球菌脑膜炎4例。21.6%(19/88)的病人死于非AIDS相关疾病,死因主要为非AIDS相关肿瘤11例,包括肺癌、直肠癌、肝癌及白血病等,其次为乙型肝炎或丙型肝炎相关肝硬化5例。死亡病人基线平均CD4~+T淋巴细胞(简称CD4细胞)计数为(107±90)个/μL,其中,基线CD4细胞计数200个/μL占78.4%。死亡时平均CD4细胞计数为(171±124)个/μL。结论本院抗病毒治疗病人总死亡率较低,超过一半的死亡病人死因仍为AIDS相关疾病,免疫重建较好的病人主要死亡原因为非AIDS相关疾病。     

1010例脑血管疾病患者死因分析

目的分析我院2006年至2016年10年间住院脑血管疾病死亡病例的死因构成及分布规律,为提高脑血管疾病的防治水平提供参考。方法将10年期间本院死亡的脑血管疾病患者1010例纳入研究,收集整理其死亡资料后进行回顾性分层分析。结果在1010例死亡案例中,其根本死因为缺血性脑卒中376例(37.23%)、出血性脑卒中325例(32.18%)、未分类脑卒中53例(5.25%)、脑卒中后遗症256例(25.35%)。首位直接死因以原发脑血管疾病(50.79%),肺部疾病(33.17%)和循环系统疾病(7.62%)为主。脑血管疾病年死亡人数呈明显上升趋势(APC=5.94%,P0.01)。男女性死亡数在死亡时年龄、婚姻状况、文化程度、根本死因的分布上差异均有统计学意义(P0.05)。在根本死因中,男性死于出血性脑卒中、脑卒中后遗症的分布多于女性,而缺血性脑卒中的分布少于女性;在首位直接死因中,男性死于神经系统疾病、呼吸系统疾病的分布多于女性,而死于循环系统疾病的分布少于女性。结论脑血管疾病死亡患者数呈明显上升趋势,根本死亡原因的分布均存在性别差异。优化医疗资源配置,针对不同脑血管疾病患者人群中高致死率的疾病,加强防控有助于提高老年人的健康水平。     

急性白血病并发出血及其治疗

出血是急性白血病(AL)的常见症状和主要死因之一.在未并发DIC的病例,出血发生率约为67～75%,其中死于出血者占38～44%;在并发DIC的病例,几乎全有出血倾向,其中死于DIC者占20～25％.因此,研究AL的出血原因及防治措施,对提高缓解率及降低死亡率有重要意义. 出血的原因     

系统性红斑狼疮患者死亡原因分析

目的 了解系统性红斑狼疮(SLE)患者主要死因的变化,分析影响SLE患者病死率和主要死因的因素.方法 回顾分析1986年1月至2006年11月北京协和医院SLE住院患者的死亡病例.结果 近21年SLE的住院患者共2558例,死亡患者195例,其中女性171例,男性24例,女性SLE病死率7.7%,男性SLE病死率7.1%,总病死率7.6%;感染、狼疮脑病和狼疮肾炎(LN)为SLE最常见的主要死因,分别占总死亡人数的30.8%、21.5%和17.4%.SLE发病后第1年内死亡人数最多,占总死亡人数的31.3%,狼疮脑病和感染是发病后第1年内最常见的死因;狼疮脑病患者主要死于发病早期,发病3年内狼疮脑病的死亡人数占狼疮脑病总死亡人数的73.8%;LN多死于狼疮发病10年以后,占LN总死亡人数的32.4%.近21年住院SLE患者的病死率逐渐下降,由1986年至1990年的12.8%降至2001年至2006年11月的5.3%;1986年至1995年SLE最常见的死因为LN,占总死亡人数的29.7%;1996年至2006年11月最常见的死因为感染,占总死亡人数的33.6%.结论 感染、狼疮脑病和LN是SLE的最主要死因,SLE发病第1年内死亡最多见.SLE患者的病死率呈逐渐下降的趋势.1986年至1995年SLE最常见死因为LN,1996年至2006年11月SLE最常见死因为感染.     

急性白血病中的感染问题

感染是急性白血病过程中的常见并发症,也是引起死亡的主要原因之一。我院从1959年至1972年的217例急性白血病中,132例(60.8％)的致死原因是感染。近年来,由于急性白血病在化疗方面取得了重大的进展,感染的预防和处理更成为提高该病缓解率和延长缓解期的一个突出问题。现综合部分国外资料如下。发生率 Viola于1967年分析了70例急性白血病的死亡原因,感染占37～40％,出血占27～36％。Schimpff等于1972年报告48例急性非淋巴细胞性白血病,发生感染111次,其中40次伴菌血症。在30个月中有43例死亡,70％(30例)死于感染。Levine等在1972年分析了450例急性白血病(包括少数再生障碍性贫     

大剂量阿糖胞苷巩固治疗核心结合因子相关急性髓系白血病的疗效及安全性分析

目的:探讨大剂量阿糖胞苷(HDAraC)巩固治疗核心结合因子相关急性髓系白血病(CBF-AML)的疗效和安全性。方法:回顾分析本院血液科骨髓移植病区2007年至2012年收治的CBF-AML患者应用HDAraC巩固化学治疗(化疗)的临床资料。评估患者总生存(OS)率、无病生存(DFS)率及复发率,并评价其治疗安全性,包括血液学不良反应及胃肠道、中枢神经系统等非血液学不良反应。结果:入选14例患者(男9例、女5例),中位年龄36.5(15~58)岁;AML-M2b 7例,AML-M4Eo 7例。共进行38个疗程HDAraC治疗。中位随访32.3个月,预期5年OS率为67.4%±14.0%,5年DFS率为44.6%±14.4%,5年复发率56.3%±14.4%。截止末次随访,10例患者存活,1例死于血小板减少所致颅内出血,3例死于疾病复发。7例患者复发,其中1例为复杂染色体异常,2例AML-M2b伴c-kit基因突变。29个疗程可评价不良反应:所有患者均出现Ⅳ度粒细胞减少和Ⅳ度血小板降低以及粒细胞缺乏性发热,经治疗无严重感染致死病例,1例因血小板减少致颅内出血死亡。患者均有Ⅰ~Ⅱ度胃肠道不良反应。未见中枢神经系统、皮肤等不良反应。结论:HDAraC应用于CBF-AML缓解后巩固化疗的效果和安全性令人满意,值得临床推广应用。     

急性重症狼疮性肾炎死因分析

目的探讨急性重症狼疮性肾炎(LN)的死因及其防治措施.方法对急性重症LN死亡患者12例进行回顾性分析.结果12例透析治疗的同时,给予大剂量甲基强的松龙及环磷酰胺联合冲击治疗.死于感染6例(占50%),心衰2例,电解质紊乱2例,急性呼吸窘迫综合征1例,狼疮性脑炎1例.结论急性重症LN通过积极治疗均非死于急性肾功能衰竭,而死因以感染为主,与患者全身多脏器损害,免疫功能低下及免疫抑制剂使用不当有关,此外,需提高对狼疮性心肌炎及纠正电解质紊乱的认识.     

Central nervous system involvement at first relapse in patients with acute promyelocytic leukemia treated with all-trans retinoic acid and anthracycline monochemotherapy without intrathecal prophylaxis

Background

The prevalence of and risk factors for central nervous system recurrence in patients with acute promyelocytic leukemia are not well established and remain a controversial matter.

Design and Methods

Between 1996 and 2005, 739 patients with newly diagnosed acute promyelocytic leukemia enrolled in two consecutive trials (PETHEMA LPA96 and LPA99) received induction therapy with all-trans retinoic acid and idarubicin. Consolidation therapy comprised three courses of anthracycline monochemotherapy (LPA96), with all-trans retinoic acid and reinforced doses of idarubicin in patients with an intermediate or high risk of relapse (LPA99). Central nervous system prophylaxis was not given.

Results

Central nervous system relapse was documented in 11 patients. The 5-year cumulative incidence of central nervous system relapse was 1.7% (LPA96 3.2% and LPA99 1.2%; p=0.09). The cumulative incidence was 0%, 0.8%, and 5.5% in low-, intermediate-, and high-risk patients, respectively. Relapse risk score (p=0.0001) and the occurrence of central nervous system hemorrhage during induction (5-year cumulative incidence 18.7%, p=0.006) were independent risk factors for central nervous system relapse.

Conclusions

This study shows a low incidence of central nervous system relapse in patients with acute promyelocytic leukemia following therapy with all-trans retinoic acid and anthracycline without specific central nervous system prophylaxis. Central nervous system relapse was significantly associated with high white blood cell counts and prior central nervous system hemorrhage, which emerged as independent prognostic factors.     

Central nervous system involvement in acute nonlymphocytic leukemia. A prospective study of adults in remission

To identify adults with acute nonlymphocytic leukemia at risk for the development of central nervous system involvement, we performed periodic cerebrospinal fluid examinations on patients in remission. Among 58 consecutive patients monitored during first remission, central nervous system leukemia developed in nine (16 percent). Four patients, including one who was symptomatic, had central nervous system leukemia detected simultaneously with marrow relapse. Five additional patients were asymptomatic and continue to have bone marrow remission. Following central nervous system and systemic treatment, two of these five patients have never had relapse, and three had relapse in the bone marrow five, 10, and 21 months later. Factors at diagnosis associated with the subsequent development of central nervous system leukemia were elevated leukocyte count, serum lysozyme and lactate dehydrogenase, extramedullary infiltration including splenomegaly, and monocytic (FAB M4 or M5a) morphology. In six of 17 patients (35 percent) with monocytic morphology, central nervous system leukemia developed compared with only three of 41 patients (7 percent) with other subtypes (p = 0.02). Discriminant analysis identified leukocyte count, splenomegaly, and M4 or M5a morphology as the most important risk factors and led to a mathematical formula that correctly identified 90 percent of the patients. Although the risk of central nervous system leukemia in adults with acute nonlymphocytic leukemia is too low to justify routine prophylaxis, those patients recognized to be at a greater risk should receive prophylaxis or be monitored closely with periodic lumbar punctures.     

Extramedullary relapse in acute leukemia

Thirty four cases of isolated extramedullary relapse after complete remission of acute leukemia were reported. The relapse took place in central nervous system (CNS), genito-urinary system, skin, serous cavity, tonsils, lymph nodes, liver, spleen, gastro-intestinal tract, pancreas and ocular fundus. These 34 cases of isolated extramedullary relapse of leukemia was found in 206 cases with complete remission, constituting 16.5% of the total. In order to discover CNS relapse with no symptoms and signs, the author strongly suggest that cerebrospinal fluid examination be carried out periodically. If some symptoms signs in the patient can not explained by infection, congestion or other causes, extramedullary relapse should be considered and further examination and appropriate treatment are needed.     

Causes of death in adults with acute leukemia.

The causes of death were investigated in 315 adults with acute leukemia during a 7-year period (1966-1972). Infection alone or in combination was the most common cause (75%), followed by hemorrhage (24%) and organ failure (9%). Most of the infections were either systemic or pulmonary. Seventy-five percent of the systemic infections and 72% of the pneumonias were caused by bacteria. Klebsiella pneumoniae, Escherichia coli and Pseudomonas aeruginosa were the most frequent organisms isolated. After 1968, there was a sharp decrease in the number of fatal infections caused by Pseudomonas aeruginosa and a marked increase in the incidence of fatal infections caused by Klebsiella spp. and E. coli. Infections caused by Gram-positive cocci occurred in only 3% of the cases. The incidence of systemic fungal infections was 13%; most common fungi causing infection were Candida spp. and Aspergillus spp. Eighty-five percent of 159 patients with a terminal neutrophil count of less than 100/mm3 died of infection, compared to 48% of 62 patients with a terminal neutrophil count of greater than 1000/mm3. Hemorrhage was mostly due to thrombocytopenia (61%) and disseminated intravascular coagulation (12%). This study indicates that infection continues to be the most common cause of death in patients with acute leukemia. Although advances in antibiotic therapy have changed the distribution of causative organisms, ultimate control of infection requires further improvements in supportive care measures which rectify impairments in the patients' host defense mechanisms.     

225例老年人猝死尸检分析

本文分析了我国２２所医学院校或医院病理室（科）或法医系登记的１９５０年至１９８９年间６０岁以上猝死尸检病例２２５例，男性１３７例，女性８８例，男女比为１．５∶１。老年人猝死的原因主要是心血管系统疾病（４９．８％），中枢神经系统疾病（３０．６％），消化系统疾病（１１．１％），而呼吸系统疾病较少（仅３．６％）。最常见的几种疾病依次是冠心病、脑出血、特发性高血压、急性胰腺炎和原因不明者。     

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急性白血病(AL)中枢神经系统受累,称中枢神经系统白血病(CNSL),发生在AL任何时期,以急性淋巴细胞白血病(ALL)居高。初发时高白细胞计数、有明显髓外侵润、某些类型如M4、M5a、T细胞型、成熟Burkitt型、复发M3及有t(4;11)、Ph+遗传学特征都是CNSL高危发病因素。脑脊液(CSF)涂片中找到白血病细胞最具诊断意义。CSF白细胞>5个/μL,伴有CNS异常表现即可诊断CNSL。对CSF进一步分类有助于ALL分层治疗。CNSL治疗重在预防,常用的防治方法有鞘内化疗、全身系统治疗以及放射治疗。现代治疗模式不再强调放疗的作用,中大剂量化疗药物的早期使用可有效防治CNSL。     

Causes of death in Korean patients with systemic lupus erythematosus: a single center retrospective study.

OBJECTIVE: To determine the causes of death in Korean patients with systemic lupus erythematosus (SLE). METHODS: We evaluated retrospectively Korean SLE patients who were monitored in the Center for Rheumatic Disease in Kang-Nam St. Mary's Hospital from 1993 to 1997 and who died. RESULTS: Forty-three (7.9%) of 544 patients died. Comparison of demographics and disease activity indices between the deceased and the survivors showed that the age was older and C3 at presentation was lower in the deceased (n = 40) than the survivors (n = 453) (age: 33.8 +/- 13.6 versus 28.3 +/- 10.6 years, p = 0.02, C3: 36.8 +/- 21.4 versus 49.7 +/- 20.8 mg/dl, p = 0.03). Among 40 patients who died, the frequency and causes of death were as follows: 13 from infection (32.5%), 10 SLE-related factors (25.0%), 6 pulmonary hypertension (15.0%), 4 cerebrovascular accidents (10.0%), and 3 thrombotic thrombocytopenic purpura (7.5%). The majority of the SLE-related deaths were non-renal in origin, including 3 cerebral nervous system disease, 2 TTP, 2 acute pulmonary hemorrhage syndrome, 1 acute myocarditis, and 1 multi-system illness. SLE-related renal causes were responsible for only death. The major organisms of infection were gram negative bacilli (69.2%), primarily manifesting as sepsis or bacteremia (76.9%). The patients (n = 13) who died from infection had lower levels of complement and higher levels of anti-ds DNA antibody at presentation than those (n = 27) who died from the other causes (C3: 24.7 +/- 17.8 versus 41.7 +/- 21.5 mg/dl, p = 0.02, anti-dsDNA antibody: 68.0 +/- 73.5 versus 27.0 +/- 35.3 IU, p = 0.04). The mean steroid dose being administered one month before death was also higher in the patients who died of infection (30.5 +/- 15.2 versus 15.2 +/- 7.7 mg/day, p = 0.03). Patients who died of pulmonary hypertension, the third most common cause of mortality, showed extremely high pulmonary pressures at the initial diagnosis, with a short interval to death, and had less major organ involvement at death. There were no deaths due to coronary heart disease or neoplasm in this cohort. CONCLUSION: The most common cause of death in 544 Korean lupus patients was infection, mainly manifesting as gram negative bacterial sepsis. SLE-related factors (mostly non-renal) were the next most frequent cause. Death from infection was associated with higher disease activity at presentation and a higher dose of steroid used previously. Death due to pulmonary hypertension was common, whereas death due to coronary heart disease was absent.     

Causes of death in sickle cell disease: an autopsy study

More precise analysis of causes of death is needed to focus research efforts and improve morbidity and mortality in sickle cell disease. In this study, the morphological evidence of the cause of death was studied in 306 autopsies of sickle cell disease, which were accrued between 1929 and 1996. The most common cause of death for all sickle variants and for all age groups was infection (33-48%). The terminal infection was heralded by upper respiratory tract syndromes in 72.6% and by gastroenteritis in 13.7%. The most frequent portal of entry in children was the respiratory tract but, in adults, a site of severe chronic organ injury. Other causes of death included stroke 9.8%, therapy complications 7.0%, splenic sequestration 6.6%, pulmonary emboli/thrombi 4.9%, renal failure 4.1%, pulmonary hypertension 2.9%, hepatic failure 0.8%, massive haemolysis/red cell aplasia 0.4% and left ventricular failure 0.4%. Death was frequently sudden and unexpected (40.8%) or occurred within 24 h after presentation (28.4%), and was usually associated with acute events (63.3%). This study shows that the first 24 h after presentation for medical care is an especially perilous time for patients with sickle cell disease and an acute event. Close monitoring and prompt aggressive treatment are warranted.     

45例初治高白细胞性急性白血病的治疗效果与预后分析

目的:探讨初治高白细胞急性白血病(HAL)的临床特征、治疗效果及预后分析。方法:对45例初治HAL进行临床回顾性分析,同时以371例非高白细胞急性白血病(NHAL)作对照组。结果:HAL构成比为10.8%。HAL组的髓外浸润、DIC、白细胞淤滞综合征及早期死亡率较对照组高。HAL组完全缓解(CR)率为28.9%,低于NHAL的57.1%,早期病死率是17.8%。早期死亡的主要原因是颅内出血和呼吸窘迫综合征。HAL的缓解率和早期死亡率与FAB分型有关。结论:HAL完全缓解率低,早期死亡率高,预后差,特别是HAL-M3的早期死亡率高,要重视其早期处理。HAL的早期积极治疗可降低早期死亡率。     

Cerebrovascular disease in acute leukemia: a clinicopathological study of 14 patients

OBJECTIVE: Cerebrovascular disease (CVD) is a serious complication of acute leukemia, and the underlying conditions are different from the common risk factors for CVD. The aim of this study was to characterize the clinical and pathological features of CVD in patients with acute leukemia. PATIENTS OR MATERIALS: In our series of 116 autopsied cases of acute leukemia during the period between January 1978 and December 1998, we had 14 patients who had CVD during the course of acute leukemia. The neuropathological and clinical features of those patients were examined. RESULTS: Neuropathological examination showed hemorrhagic infarction due to disseminated aspergillosis or mucormycosis (5 cases), multiple hemorrhages due to leukemic cell infiltration (2 cases) and a single massive hemorrhage with petechial hemorrhages in various regions of the brain (4 cases). Three patients had CVD due to miscellaneous causes. Clinicopathological correlation revealed that fungal disseminations occurred under agranulocytosis, while leukemic cell infiltration occurred under a marked leukocyotosis (peripheral white blood cell count >100,000/microl). Four patients with coagulopathy, including three with disseminated intravascular coagulation (DIC) had a single massive hemorrhage. CONCLUSION: Our study demonstrated that there were at least three types of CVD with specific clinicopathological features. Hemorrhagic infarction under agranulocyotosis was due to disseminated aspergillosis or mucormycosis. Multiple cerebral hemorrhages under marked leukocyotosis was due to leukemic cell infiltration into the brain. Massive cerebral hemorrhage was associated with coagulopathy including DIC.