Castleman病患者的临床特征及预后分析

目的：分析Castleman病的临床特征及预后，以提高Castleman病的诊治水平。方法：回顾性分析甘肃省人民医院自2009年1月至2020年11月经病理活检诊断为Castleman病的29例患者的临床资料。根据临床分型分为两组：单中心型组（UCD,n=20）和多中心型组（MCD,n=9），对其临床表现、实验室检查、治疗方案、病理学检查和随访资料进行统计学分析。结果：UCD组和MCD组平均年龄、男女比例的差异无统计学意义。UCD患者中透明血管型占比80.0%，浆细胞型占比20.0%;MCD患者中透明血管型占比33.3%；浆细胞型占比55.6%，混合型占比11.1%，两组病理分型差异有统计学意义（P=0.039）。UCD患者常表现为无症状的单个淋巴结肿大，临床症状轻微；MCD患者表现为全身多发浅表及深部淋巴结肿大。MCD组乏力、脾大、浆膜腔积液发生率均高于UCD组（P<0.05），同时，贫血、低蛋白血症、血沉升高、血清球蛋白升高、β2-微球蛋白升高发生率均明显高于UCD组（P<0.05）。而两组白细胞计数和血小板计数异常、乳酸脱氢酶升高发生率相比较，差异均没有统计学意义（P...     

Castleman病合并贫血的临床分析

目的:本研究旨在探讨Castleman病(CD)合并贫血的特征。方法:回顾性研究33例CD患者的临床资料,分析贫血发生率和临床特征,探讨贫血类型及其治疗和疗效。结果:13/33(39%)例CD初诊时有贫血表现,多为轻度,正细胞正色素型。多中心CD(multicentric CD,MCD)贫血发生率高于单中心CD(unicentric CD,UCD)(85%vs 10%)(P0.001)。与无贫血者相比,CD合并贫血者多具有系统性表现,且血沉、炎症指标(C反应蛋白和纤维蛋白原)水平、多克隆免疫球蛋白增多及自身抗体阳性的发生率均升高(P均0.05)。进一步分析贫血类型发现,除2例自身免疫性溶血性贫血(AIHA)和1例脾亢继发贫血外,余10例均具有慢性病贫血(ACD)的特征,其血红蛋白水平与C反应蛋白(CRP)及纤维蛋白原均呈负相关(r分别为-0.917、-0.717,P均0.001)。UCD合并贫血者经肿瘤切除后治愈,MCD合并贫血者经免疫和/或化疗治疗后贫血好转。结论:贫血为MCD常见的系统性表现,亦可见于UCD。除少数表现为AIHA外,多数具有ACD特征。CD合并贫血的治疗以治疗原发病为主。     

罕见肺内原发Castleman病2例病理特征及文献复习

<正>Castleman病(Castleman disease, CD),也称巨淋巴结增殖症，1956年由Castleman等[1]首先报道并命名，是一种非干酪性淋巴结增殖性病变。根据病变累及范围及临床表现分为：单中心型(UCD)和多中心型(MCD),根据病理学改变可分为透明血管型(HV-CD)、浆细胞型(PC-CD)和混合型，其中HV-CD多见[2],UCD常发生在纵隔或者颈部淋巴结，侵犯结外或者肺实质内比较罕见，罕有文献报道，     

30例不同类型胸部受累Castleman病临床分析

目的总结不同类型胸部受累Castleman病(CD)的临床、影像学、病理及诊治特征,以提高临床医师对CD的认识。方法回顾性分析广州医科大学附属第一医院自2009年6月至2019年5月收治入院的胸部受累CD患者30例,将其分为闭塞性细支气管炎(BO)组、不伴BO的单中心型Castleman病(UCD)组及不伴BO的多中心型Castleman病(MCD)组,分析其临床资料并总结其特征。结果30例患者中,5例(16.7%)纳入BO组,18例(60.0%)纳入不伴BO的UCD组,7例(23.3%)纳入不伴BO的MCD组。不伴BO的MCD组中位年龄明显大于BO组[(49.29±5.39)岁对(27.20±3.76)岁,P=0.005]和不伴BO的UCD组[(49.29±5.39)岁对(37.17±2.87)岁,P=0.034]。胸部症状在BO组(100%)和不伴BO的MCD组(71.4%)多见,而不伴BO的UCD组无胸部症状。贫血及IgG增高仅在不伴BO的MCD组出现(发生率均为57.1%),红细胞沉降率增快及低氧血症在BO组(发生率分别为40.0%和60.0%)及不伴BO的MCD组(发生率分别为57.1%和28.6%)均可出现。BO组患者的肺功能均表现为极重度混合性通气功能障碍。胸部CT所示肺实质受累率:BO组为100%;不伴BO的MCD组为57.1%,表现为双肺弥漫性病变;不伴BO的UCD组为11.1%,表现为孤立性肺结节。不伴BO的MCD组的淋巴结短径明显小于BO组[(1.83±0.51)cm对(4.73±1.63)cm,P=0.006]和不伴BO的UCD组[(1.83±0.51)cm对(3.62±0.26)cm,P=0.011]。BO组病理类型均为透明血管型(100%),不伴BO的UCD组88.9%为透明血管型,而不伴BO的MCD组以浆细胞型为主(57.1%)。BO组患者均出现口腔溃疡,在肿物切除术后予免疫调节剂治疗,溃疡症状缓解,但肺部症状仍进行性加重。不伴BO的UCD组主要治疗方式为胸腔镜下肿物切除,不伴BO的MCD组主要治疗方式为化疗、免疫调节剂、靶向治疗等。结论三组患者的年龄、临床症状、实验室检查、肺功能、影像学表现、病理类型、治疗及预后均有所不同,此分类能促进临床医师对本病的认识。     

Castleman病的诊治(附8例报告)

目的探讨Castleman病的临床表现及外科治疗。方法对我院自2003年6月起收治的8例Castleman病患者的临床资料进行分析。结果Castleman病临床分为局灶型(LCD)和多中心型(MCD),LCD多表现为单发淋巴结肿大并周围卫星病灶,而MCD表现为全身多处淋巴结肿大。LCD7例行手术切除,MCD1例行手术活检,所有病例均经病理明确诊断。LCD手术切除疗效好。结论手术切除是LCD的主要治疗手段,仔细分离、结扎血管及剔除淋巴滤泡是手术的关键。而对于MCD,手术活检有助于明确诊断及指导进一步治疗。     

多中心型Castleman病1例漏诊分析

Castleman（CD）病又称巨大淋巴结增生症，是原因未明的淋巴结增生性疾病，临床上较为少见，其临床表现多样且无特异性，诊断较困难，为了提高对CD病的认识，减少临床漏诊和误诊，本文报道1例多系统受累的多中心型CD病，并复习文献就CD病的临床和实验室检查和病理学特点以及相应的诊治措施进行讨论。CD病的临床类型分为局灶型和多中心型（全身性），病理类型有透明血管型（HV型）、浆细胞型（PC型）。早期确诊主要依靠组织病理学检查，淋巴结滤泡内和滤泡间淋巴组织增生，滤泡周围的小淋巴细胞围绕滤泡中心呈环层状排列似葱皮样结构，滤泡间血管增生明显，有血管增生样玻璃样变，是透明血管型的病理组织学特征。     

Castleman病60例临床病例分析

目的:总结Castleman病(Castlemans disease,CD)的临床特点。方法:对2003-2016年在本院诊断Castleman病的60例患者的临床资料做回顾性分析。结果:60例CD患者根临床分为局灶型(unicentric CD,UCD)和多中心型(multicentric CD,MCD),分别包括三个病理类型:透明血管型(hyaline vascular type,HV-CD)、浆细胞型(plasma cell type,PC-CD)、混合型(mixed type,Mix-CD)。37例UCD临床症状轻微,均行手术治疗为主,预后较好。23例MCD临床症状复杂,其中8例有水肿或胸、腹水,6例体重减轻或乏力,5例出现咳嗽、咳痰,4例有心悸、气促,4例有口腔溃疡,3例有皮肤溃疡,3例有发热,3例有出血症状,2例出现肢体、皮肤麻木,盗汗,消化道症状和肝脾肿大各1例;实验室检查可见免疫球蛋白升高、自身抗体以及类风湿因子升高、急性期反应物升高、低白蛋白血症、贫血等异常;需手术和药物综合治疗,预后较差。结论:Castleman病可通过病理活检确诊,UCD临床症状轻微,手术治疗效果好,MCD临床症状复杂,需综合治疗,预后相对较差。     

多中心型Castleman病的研究进展

Castleman病,又称为巨大淋巴结增生症或者血管淋巴性滤泡组织增生,该病为一种少见的异质性淋巴组织增生性疾病.目前,对Castleman病的文献报道多为小样本量或者个案研究,而研究主要集中于多中心型Castleman病(MCD)的发病机制及其治疗方面,并且取得了一些新的进展.笔者拟就MCD的病因及发病机制、临床表现、诊断及治疗等方面的研究进展进行综述.     

系统性红斑狼疮患者CRP水平的分析及临床意义

目的 探讨系统性红斑狼疮（SLE）患者C-反应蛋白（CRP）水平变化及临床意义.方法 回顾性分析2008年8月～2010年8月苏州大学附属第一医院风湿科住院的205例SLE患者,按影像学有无浆膜腔积液表现将其分为有、无浆膜腔积液组,按SLEDAI评分分为有、无活动两组,按统一法获得各观察参数指标数据,分析CRP水平与SLE活动性及浆膜腔积液的关系,并比较分析ESR、C4、C3、IgG与浆膜腔积液的关系.结果 通过分析205例SLE患者,有积液组的CRP水平明显高于无积液组（P〈0.001）,活动组的CRP水平与非活动组无明显差异（P〉0.05）,ESR、C4、C3、IgG与浆膜腔积液无相关（P〉0.05）,通过Logistic回归分析,CRP水平升高与浆膜腔积液的存在有显著相关性（OR= 86.04,95%CI17.85～373,P〈0.001）.结论 SLE患者的CRP水平在反映疾病活动性方面意义不大,但CRP水平升高与浆膜腔积液的存在显著相关.     

巨大淋巴结增生症肺部CT表现

目的 探讨巨大淋巴结增生症（CD）患者肺部病变的CT特征。方法 收集14例肺部有异常表现的CD患者,包括多中心型（MCD）8例,单中心型（UCD）6例,对其肺部CT表现进行回顾性分析。结果 3例肺门UCD表现为肺门单发均质等密度类圆形肿物,增强后均质明显或显著强化;1例腹膜后UCD合并副肿瘤性天疱疮,表现为两肺闭塞性细支气管炎;2例纵隔UCD两肺见多发边缘清晰、小叶中心分布小结节。8例MCD均以两肺多发小结节为主,2例见最大径为1~2 cm大结节;4例结节边缘模糊,4例边缘清晰;小结节以小叶中心分布为主,其中3例见少许小结节分布于胸膜下。其他征象包括小叶间隔增厚4例,磨玻璃密度影4例,索条影4例,斑片状实变3例,囊腔2例,血管支气管束增粗1例。结论 CD肺部CT表现复杂多样,可能与病理分型有关。     

A novel prognostic model for angioimmunoblastic T-cell lymphoma: A retrospective study of 55 cases

ObjectiveTo explore prognostic factors and develop an accurate prognostic prediction model for angioimmunoblastic T-cell lymphoma (AITL).MethodsClinical data from Chinese patients with newly diagnosed AITL were retrospectively analysed. Overall survival (OS) and progression-free survival (PFS) were estimated using Kaplan-Meier method survival curves; prognostic factors were determined using a Cox proportional hazards model. The sensitivity and specificity of the predicted survival rates were compared using area under the curve (AUC) of receiver operating characteristic (ROC) curves.ResultsThe estimated 5-year OS and PFS of 55 eligible patients with AITL were 22% and 3%, respectively. Multivariate analysis showed that the presence of pneumonia, and serous cavity effusions at initial diagnosis were significant prognostic factors for OS. Based on AUC ROC values, our novel prognostic model was superior to IPI and PIT based models and suggested better diagnostic accuracy.ConclusionsOur prognostic model based on pneumonia, and serous cavity effusions at initial diagnosis enabled a balanced classification of AITL patients into different risk groups.     

Prognostic nomograms for predicting overall survival and cause-specific survival of signet ring cell carcinoma in colorectal cancer patients

BACKGROUNDSignet ring cell carcinoma (SRCC) is an uncommon subtype in colorectal cancer (CRC), with a short survival time. Therefore, it is imperative to establish a useful prognostic model. As a simple visual predictive tool, nomograms combining a quantification of all proven prognostic factors have been widely used for predicting the outcomes of patients with different cancers in recent years. Until now, there has been no nomogram to predict the outcome of CRC patients with SRCC.AIMTo build effective nomograms for predicting overall survival (OS) and cause-specific survival (CSS) of CRC patients with SRCC.METHODSData were extracted from the Surveillance, Epidemiology, and End Results database between 2004 and 2015. Multivariate Cox regression analyses were used to identify independent variables for both OS and CSS to construct the nomograms. Performance of the nomograms was assessed by concordance index, calibration curves, and receiver operating characteristic (ROC) curves. ROC curves were also utilized to compare benefits between the nomograms and the tumor-node-metastasis (TNM) staging system. Patients were classified as high-risk, moderate-risk, and low-risk groups using the novel nomograms. Kaplan-Meier curves were plotted to compare survival differences.RESULTSIn total, 1230 patients were included. The concordance index of the nomograms for OS and CSS were 0.737 (95% confidence interval: 0.728-0.747) and 0.758 (95% confidence interval: 0.738-0.778), respectively. The calibration curves and ROC curves demonstrated good predictive accuracy. The 1-, 3-, and 5-year area under the curve values of the nomogram for predicting OS were 0.796, 0.825 and 0.819, in comparison to 0.743, 0.798, and 0.803 for the TNM staging system. In addition, the 1-, 3-, and 5-year area under the curve values of the nomogram for predicting CSS were 0.805, 0.847 and 0.863, in comparison to 0.740, 0.794, and 0.800 for the TNM staging system. Based on the novel nomograms, stratified analysis showed that the 5-year probability of survival in the high-risk, moderate-risk, and low-risk groups was 6.8%, 37.7%, and 67.0% for OS (P < 0.001), as well as 9.6%, 38.5%, and 67.6% for CSS (P < 0.001), respectively.CONCLUSIONConvenient and visual nomograms were built and validated to accurately predict the OS and CSS rates for CRC patients with SRCC, which are superior to the conventional TNM staging system.     

Factors associated with overall survival in early gastric cancer patients who underwent additional surgery after endoscopic submucosal dissection

BACKGROUNDControversy exists about the benefit of additional surgery after endoscopic submucosal dissection (ESD) for early gastric cancer (EGC).AIMTo examine risk factors for overall survival (OS) after additional surgery in patients with EGC who initially underwent ESD.METHODSThis was a retrospective analysis of patients with EGC who underwent additional surgery after ESD at the Beijing Friendship Hospital affiliated to Capital Medical University between August 2012 and August 2019. OS was the primary outcome. Lymph node metastasis and residual tumor were secondary outcomes. Logistic regression models and Kaplan-Meier curves were used for further analysis. RESULTSForty-two patients were evaluated, including 35 (83.3%) males and 7(16.7%) females. The mean age was 62 (range, 32-82) years. Male sex [hazard ratio (HR) = 21.906, 95% confidence interval (CI): 3.762-229.250; P = 0.039), T1b invasion (HR = 3.965, 95%CI: 1.109-17.432; P = 0.047), undifferentiated tumor (HR = 9.455, 95%CI: 0.946-29.482; P = 0.049), lymph node metastasis (HR = 2.126, 95%CI: 0.002-13.266; P = 0.031), and residual tumor (HR = 4.275, 95%CI: 1.049-27.420; P = 0.043) were independently associated with OS. The follow-up duration was 4-81 mo (median: 50.7 mo). OS was 77.0 ± 12.1 mo (95%CI: 53.3-100.7 mo). The 3-year and 5-year OS rates were 94.1% and 85%, respectively.CONCLUSIONMale sex, T1b invasion, undifferentiated tumor, lymph node metastasis, and residual tumor are independently associated with OS in patients with EGC who underwent additional surgery after ESD.     

Development and validation of a prognostic nomogram model for Chinese patients with primary small cell carcinoma of the esophagus

BACKGROUNDPrimary small cell carcinoma of the esophagus (PSCE) is a highly invasive malignant tumor with a poor prognosis compared with esophageal squamous cell carcinoma. Due to the limited samples size and the short follow-up time, there are few reports on elucidating the prognosis of PSCE, especially on the establishment and validation of a survival prediction nomogram model covering general information, pathological factors and specific biological proteins of PSCE patients.AIMTo establish an effective nomogram to predict the overall survival (OS) probability for PSCE patients in China.METHODSThe nomogram was based on a retrospective study of 256 PSCE patients. Univariate analysis and multivariate Cox proportional hazards regression analysis were used to examine the prognostic factors associated with PSCE, and establish the model for predicting 1-, 3-, and 5-year OS based on the Akaike information criterion. Discrimination and validation were assessed by the concordance index (C-index) and calibration curve and decision curve analysis (DCA). Histology type, age, tumor invasion depth, lymph node invasion, detectable metastasis, chromogranin A, and neuronal cell adhesion molecule 56 were integrated into the model.RESULTSThe C-index was prognostically superior to the 7^th tumor node metastasis (TNM) staging in the primary cohort [0.659 (95%CI: 0.607-0.712) vs 0.591 (95%CI: 0.517-0.666), P = 0.033] and in the validation cohort [0.700 (95%CI: 0.622-0.778) vs 0.605 (95%CI: 0.490-0.721), P = 0.041]. Good calibration curves were observed for the prediction probabilities of 1-, 3-, and 5-year OS in both cohorts. DCA analysis showed that our nomogram model had a higher overall net benefit compared to the 7^th TNM staging .CONCLUSIONOur nomogram can be used to predict the survival probability of PSCE patients, which can help clinicians to make individualized survival predictions.     

Hand-assisted laparoscopic versus open surgery for radical gastrectomy in the treatment of advanced distal gastric cancer: long-term overall and disease-free survival (final results of a single-center study)

ObjectiveTo compare the surgical effects and long-term efficacy of hand-assisted laparoscopic surgery (HALS) and open surgery (OS) in radical gastrectomy for advanced distal gastric cancer.MethodsOne hundred twenty-four patients who were admitted to the Department of Gastrointestinal Surgery of the West War Zone General Hospital from May 2008 to April 2012 were randomly divided into a HALS group (n = 62) and an OS group (n = 62). After surgery, 113 patients were followed up for 5 and 8 years, and 11 patients were lost to follow-up. The 5- and 8-year overall survival and disease-free survival rates of the two groups were compared and analyzed.ResultsThe 5- and 8-year overall survival rates were 31.90% and 18.40% in the HALS group and 32.50% and 18.60% in the OS group, respectively. The 5- and 8-year disease-free survival rates were 21.50% and 13.00% in the HALS group and 21.90% and 13.10% in the OS group, respectively. No significant differences were found.ConclusionHand-assisted laparoscopic radical gastrectomy for advanced distal gastric cancer has the advantages of less severe trauma, less intraoperative blood loss, more rapid postoperative recovery, and equivalent long-term efficacy compared with OS.     

MRI morphologic and clinicopathologic characteristics for predicting outcomes in patients with locally advanced rectal cancer

Purpose

The aim of this study was to investigate the value of MRI morphologic and clinicopathologic factors for predicting 3-year disease-free survival (DFS) in patients with locally advanced rectal cancer (LARC).

Method

In this retrospective study, pre- and post-neoadjuvant chemoradiotherapy (nCRT) MRI morphologic (e.g., pre-nCRT MRI-detected extramural venous invasion) and clinicopathologic variabilities (e.g., pathological complete response) were evaluated in all patients. Three-year DFS was estimated using Kaplan–Meier product-limit method, and Cox proportional hazards models were used to determine associations between morphologic or clinicopathologic variabilities and survival outcomes.

Results

A total of 115 patients (39 females and 76 males; median age, 54 years; age range, 28–82 years) with LARC treated with nCRT were enrolled. With a median follow-up of 48.0 months, the 3-year DFS was 79.0% for all patients. During follow-up, 18 patients died, 28 patients experienced relapse (26 distant, one local, and one both), and 69 patients were censored. MRI-detected extramural venous invasion (mrEMVI) was the only significantly independent factor of long-term survival, while HR was 2.308 (95% CI 1.151–4.629, P = 0.018) on univariate and 2.495 (95% CI 1.243–5.012, P = 0.010) on multivariate analysis. The 3-year cumulative survival rate in patients with mrEMVI negativity compared with positivity were 86.6% versus 65.0% (P = 0.015), respectively.

Conclusion

In conclusion, pre-nCRT mrEMVI status was the independent significant risk factor for long-term outcomes in LARC patients treated with nCRT, while the other morphologic and clinicopathologic characteristics were not related to the patient survival.

     

The effect of comorbidity on survival and collected CD34 + cell counts in autologous hematopoietic stem cell transplant patients

ObjectiveIn this study, we aimed to report the effectiveness of hematopoietic cell transplantation–specific comorbidity index (HCT-CI) and GATMO scores in predicting overall survival (OS) who underwent autologous stem cell transplantation (ASCT).Material and methodsThe data of 263 MM and 204 lymphoma patients who underwent ASCT in the last 11 years were retrospectively analyzed.ResultsNeutrophil engraftment time, thrombocyte engraftment time and collected CD34+ cell counts were similar in MM patients with HCT-CI>2 and HCT-CI≤2 (all p>0.05). Although the estimated median OS of MM patients with HCT-CI ≤2 tended to be higher than those with HCT-CI>2, this difference was not statistically significant (52.8 vs 45 months, p=0.172). No effect of GATMO score on CD34 + count, engraftment times and OS in MM patients was detected (p>0.05). The effect of HCT-CI score on lymphoma patients was examined, it was found that the neutrophil engraftment time was longer (p=0.039) and the number of collected CD34+ cells was lower (p=0.02) in patients with HCT-CI>2 than those with HCT-CI≤2. While the estimated median OS of lymphoma patients with HCT-CI≤2 was 51.5 months, the estimated median OS of patients with HCT-CI>2 was 9.5 months (p=0.012). When lymphoma patients were divided into four groups according to their GATMO scores, the OS of the four groups was found to be different from each other (p<0.001).ConclusionHCT-CI and GATMO scores predict OS in lymphoma patients but not MM patients.     

Systematic review and meta-analysis of Chinese herbal medicine as adjuvant treatment in advanced non-small cell lung cancer patients

IntroductionWhether combining Chinese herbal medicines (CHMs) and chemotherapy to treat patients with advanced non-small-cell lung cancer (NSCLC) has clinical benefits has yet to be confirmed. A meta-analysis was performed to address the efficacy of CHM in patients with advanced NSCLC.MethodsSeven databases, including PubMed, MEDLINE, Cochrane Library, Embase, CINAHL Plus with Full Text (EBSCO), WANFANG DATA and the Chinese National Knowledge Infrastructure (CNKI), were systematically searched for available literature through March10, 2020. A meta-analysis was conducted to generate combined risk ratios(RRs) with 95 % confidence intervals (CIs) for objective response rates (ORRs), disease control rates (DCRs), and 1-year overall survival (OS) rates, and a random-effects model was used to estimate the standardized mean differences (SMDs) with 95 % CI for quality or life (QOL), median survival time (mST) and progression-free survival (PFS).ResultsReports of 14 randomized controlled trials involving 1451 patients were included in the analysis. Among them, 739 patients received CHMs, and 712 patients received chemotherapy alone. The ORR (RR = 1.37, 95 % CI [1.20‐1.58], p = .000), DCR (RR = 1.13, 95 % CI [1.07‐1.21], p = 0.000), QOL (SMD = 1.47; 95 % CI [0.30–2.64]; p = 0.014), mST (SMD = 1.62; 95 % CI [1.15‐2.08];p = .000), and 1-year OS rate (RR = 1.24, 95 % CI [1.05‐1.47], p = 0.01) were higher in patients with NSCLC who received CHMs than in those who received only chemotherapy. However, the CHM group was not found to have a higher median PFS (SMD = 1.27, 95 % CI [-0.22‐2.78], p = .095) than the chemotherapy group. Publication bias for ORR and DCR was indicated by funnel plot. For the efficacy endpoint, no evidence of a lack of robustness was found, according to the sensitivity analysis. These results must be interpreted with caution due to differences in the designs of the trials and patients’ characteristics, and also due to the presence of missing data.ConclusionsOur study found that higher ORR, DCR, QOL, mST and 1-year OS rate were associated with CHM use as an adjuvant to chemotherapy. Although these results require further confirmation, CHMs apparently have potential therapeutic value for patients with advanced NSCLC.