tbuckley/cabot-search huggingface dataset:
Combination anti-PD-1 and ipilimumab (ipi) therapy in patients with advanced melanoma and pre-existing autoimmune disorders (AD).
Authors: Lauren Julia Brown, Alison Weppler, Prachi Bhave, Clara Allayous, James Randall Patrinely, Patrick Alexander Ott, Shahneen Kaur Sandhu, Andrew Mark Haydon, Celeste Lebbe, Douglas Buckner Johnson, Georgina V. Long, Alexander M. Menzies, Matteo S. Carlino
Journal: Journal of Clinical Oncology (2020)

10026 Background: Clinical trials of immunotherapy exclude patients (pts) with pre-existing AD. While retrospective data exist regarding the efficacy and safety of single agent ipi and anti-PD1 antibodies (PD1) in pts with AD, no data are available regarding the safety and efficacy of combination therapy in pts with AD, which has a higher toxicity risk. Methods: Pts with melanoma and pre-existing AD treated with combination ipi/PD1 were retrospectively identified from 10 international centres. Data regarding AD, treatment, toxicity and outcomes were examined. Results: Fifty-five pts were included, 46 were treated with ipi/nivolumab and 9 with ipi/pembrolizumab. 40 had an ipi dose of 3mg/kg while 15 had a lower dose regimen. 9 pts received prior PD1 therapy; 3 suffered moderate immune-related adverse events (irAE) with no flares of AD on single agent PD1. Pre-existing AD included inflammatory bowel disease (IBD), thyroiditis, rheumatoid arthritis (RA), multiple sclerosis and psoriasis. 10 pts had active symptoms of AD and 13 were immunosuppressed at commencement of ipi/PD1. Eighteen pts (33%) experienced a flare of their AD including 4/7 with RA, 3/6 with psoriasis, 5/9 with IBD, 3/18 with thyroiditis, 1/1 with Sjogren’s syndrome, 1/1 with polymyalgia, 1/1 with Behcet’s syndrome. Median time to flare was 19 days (range 4 – 167). 13 pts were managed with steroids, 5 required additional immunosuppressants. 7 pts were hospitalised for management of flare (5 with IBD, 2 with RA). 2 pts required intensive care and vasopressors for severe IBD flare, quiescent prior to ipi/PD1. One for diarrhoea and shock and one for duodenal perforation. 8 pts ceased treatment due to flare (3 with IBD, 2 with RA, 1 with Behcet’s, 1 with Sjogren’s). Thirty-seven pts (67%), experienced an irAE unrelated to their AD, 38% G3 or G4. The most frequent irAEs were colitis (n = 16), hepatitis (n = 12), endocrinopathies (n = 12), with 13 pts experiencing an irAE in ≥ 2 organs. 9 pts experienced both AD flare and an irAE. 20 pts (36%) ceased immunotherapy due to irAEs. ORR was 55% (54% in PD1 naive pts), at a median follow up of 14 months, 77% of responses ongoing. ORR in pts who had a flare of their AD was 44% and in pts on immunosuppression was 46%. Median PFS was shorter in pts who had a flare of AD compared with those who did not (2.6 vs 9 months; P-value 0.047). Conclusions: Combination ipi/PD1 shows efficacy comparable to clinical trial populations in pts with pre-existing AD and advanced melanoma. Whilst there was a substantial risk of flare of AD, no increased frequency of irAE’s was observed.
Tumor response and growth rate of nivolumab treatment in advanced gastric cancer: Real-world data from a large observational/translational study, JACCRO GC-08 (deliver trial).
Authors: Ryohei Kawabata, Yasuhiro Sakamoto, Eisuke Inoue, Atsushi Ishiguro, Yusuke Akamaru, Yosuke Kito, Masazumi Takahashi, Jin Matsuyama, Hiroshi Yabusaki, Akitaka Makiyama, Takahisa Suzuki, Masahiro Tsuda, Hisateru Yasui, Hisato Kawakami, Ryo Matoba, Kei Muro, Takako Eguchi Nakajima, Wataru Ichikawa, Masashi Fujii, Yu Sunakawa
Journal: Journal of Clinical Oncology (2020)

4527 Background: Nivolumab (Nivo) demonstrated survival benefit in previously treated gastric cancer (GC) patients (pts), with a response rate (RR) of 11% and a disease control rate (DCR) of 40% (Kang YK, et al. Lancet 2017). There are few real-world data of Nivo and its predictive markers are needed in GC. It has been demonstrated that some tumors grow rapidly after Nivo treatment, but the proportion is uncertain. Methods: DELIVER trial was a prospective, multicenter, observational/translational study which assessed pts with advanced GC treated with Nivo alone and ECOG Performance Status (PS) 0-2 (UMIN000030850). The aims were to evaluate the efficacy and safety of Nivo in real world, and to discover novel host-related immune-biomarkers (gut microbiome, genetic polymorphism, gene expression, and metabolome) using fecal and blood samples which were collected before and after Nivo treatment. The RR, DCR, progression-free survival, overall survival, and tumor growth rate (TGR) were estimated as the efficacy. The response was evaluated by first imaging based on RECIST version 1.1. The TGR was calculated as a percentage increase in tumor volume during 1 month (Champiat et al. Clin Cancer Res 2017). Results: A total of 501 pts was enrolled in this study from Mar 2018 to Aug 2019, and 487 pts were evaluable for analysis (median age 70-y, 71% male, ECOG PS0/1/2 42%/44%/14%, tub/por/sig 45%/41%/5%, 21% HER2-pos, 42% pts with ascites). The DCR was 39.2% (95%CI 34.9-43.7) in evaluable pts. In 282 pts with measurable lesions, the RR was 6.7% (95%CI 4.1-10.3) and DCR was 36.5%. Sub-analysis by patient background indicated that DCR was 41% for PS0, 42% for PS1, and 24% for PS2. In addition, the DCR was lower in pts with ascites compared to those without ascites (28.6% vs. 47.0%, p= 0.005). The TGR decreased after introduction of Nivo in 124 (56.6%) of 219 evaluable pts for TGR; however, 20.5% pts were identified as experiencing hyper-progressive disease (HPD) which was defined as a ≥2-fold increase of the TGR before and after Nivo. When defining HPD as a ≥2-fold increase of tumor growth kinetics ratio and 50% increase of tumor burden, 9.6% pts experienced it. Conclusions: The real-word data of the large observational trial showed a comparable DCR to that of clinical trial in advanced GC treated with Nivo. This trial revealed the tumor behavior and some pts who experienced rapid tumor growth after Nivo treatment in clinical practice; biomarkers for HPD and the definition should be established. Clinical trial information: UMIN000030850 .
Capmatinib in patients with <i>METex14</i>-mutated or high-level <i>MET</i>-amplified advanced non–small-cell lung cancer (NSCLC): results from cohort 6 of the phase 2 GEOMETRY mono-1 study.
Authors: Harry J.M. Groen, Wallace L. Akerley, Pierre Jean Souquet, Eckart Laack, Ji-Youn Han, Egbert F. Smit, Aaron Scott Mansfield, Edward B. Garon, Juergen Wolf, Daniel Shao-Weng Tan, Rebecca Suk Heist, Maeve Waldron-Lynch, Sylvie Le Mouhaer, Ngozi Nwana, Monica Giovannini, Sergey Orlov
Journal: Journal of Clinical Oncology (2020)

9520 Background: Capmatinib (INC280) has shown promising efficacy in patients (pts) with MET exon 14 ( METex14)–mutated NSCLC who were pretreated (cohort 4) or treatment (tx)-naïve (cohort 5b) in the ongoing, multicohort, phase 2 GEOMETRY mono-1 study. We report the results for pts enrolled in the expansion cohort 6 with either high-level MET amplification (gene copy number [GCN] ≥10) or METex14 mutation (any MET GCN) whose disease progressed on 1 prior line of systemic therapy. Methods: Adult pts (≥18 years), ECOG PS 0–1 who had ALK and EGFR wt, stage IIIB/IV NSCLC (any histology) received capmatinib tablets 400 mg twice daily (with or without food). Key efficacy endpoints were overall response rate (ORR) and duration of response (DOR) by blinded independent review committee (BIRC) per RECIST v1.1. Other secondary endpoints included investigator-assessed ORR, DOR, disease control rate (DCR), progression-free survival (PFS; BIRC and investigator assessment) and safety. Results: As of Jan 6, 2020, 34 NSCLC pts with METex14 mutation (n = 31) or high-level MET amplification (n = 3) were included in this analysis. Tx was ongoing for 38.2% of pts. In METex14-mutated NSCLC pts, per BIRC assessment: ORR was 48.4%, median DOR was 6.93 months (mo, not yet mature, 95% CI: 4.17–NE) and median PFS was 8.11 mo (not yet mature, 95% CI: 4.17–9.86). Investigator-assessed responses were similar to BIRC assessment (Table). Only 3 pts with high-level MET amplification were included in this cohort due to challenges in enrollment. All 3 pts had stable disease per BIRC assessment and were on treatment for 48, 85 and 97 days. Most common AEs (≥25%, all grades, N = 34) were peripheral edema (64.7%), nausea (35.3%), fatigue (29.4%), back pain (26.5%) and vomiting (26.5%). Data for pts with brain metastasis will be presented at the ASCO 2020 meeting. Conclusions: Capmatinib was confirmed to be efficacious in 2 nd line, METex14-mutated NSCLC pts. This is the first cohort where capmatinib has been administered without fasting restriction and data confirm the favorable safety profile. Clinical trial information: NCT02414139 . [Table: see text]
Framework of organization and management of community health service institutions in Shanghai Changning district
Authors: Ping Jiang, Lei Xu, Qi Zhao, Minjie Zhu, Qiong Wu
Journal: BMJ (2017)

Objective
To explore the framework of organization and management of community health service institution in Changning district.


Methods
A questionnaire survey was conducted among 30 managers of 10 community health service centers in Changning district at October 2015. The questionnaire included the basic situation of the community health service institution, the operation of the current organization and management framework, and the intention of further adjustment.


Results
There were 5 systems (administration system, family doctor team system, inpatient system, support system and medical technical system) in current organization and management structure. Twenty seven responders (93.1%) thought that the bottlenecks and problems existed in the current organization structure (22 thought that the responsibilities of the organization and management system were unclear and overlapped; 22 thought that the cooperation and coordination between systems were less strong; 10 thought that organization systems had poor mutual understanding and consistency of action, 2 mentioned other problems). Twenty five responders (86.2%) thought that the bottlenecks and problems existed in the current management structure (18 thought that the current management model was difficult to promote reform or new work; 14 thought that there were more layers of management and less managerial efficiency; 13 thought that there was lack of effective management measures; 4 mentioned other problems). Twenty nine responders proposed suggestions dealing with the service function, management function, service organization form and new organization and management framework of community health service institutions. The family doctor studio as the core of service platform and management platform was recommended.


Conclusions
There are problems and bottlenecks in the current organization and management framework of community health service institutions in Changning district, and a new framework has been proposed according to the management recommendations.


Key words: 
Community health service; Organization and administration
Influence of lifestyle on the FAIM2 promoter methylation between obese and lean children: a cohort study
Authors: 吴丽君, 赵小元, 沈玥, 黄贵民, 张美仙, 闫银坤, 侯冬青, 孟玲慧, 刘军廷, 程红, 米杰
Journal: BMJ (2015)

目标
肥胖相关基因Fas细胞凋亡抑制分子2(FAIM2)可以被营养状况调控，并且其启动子甲基化水平与肥胖显著相关。生活行为因素，如静态生活行为和运动，可以修饰与肥胖相关的表观遗传状态。但是，FAIM2影响肥胖的分子机制是否与生活行为相关还不清楚。本研究检测了肥胖和消瘦儿童间FAIM2启动子甲基化水平与静态生活行为和运动的潜在差异。


设计
人群研究。


研究地点
首都儿科研究所。


研究对象
来自2013年北京8～18岁儿童横断面调查，包括59例肥胖儿童，并以39例消瘦儿童作为对照。


主要和次要结果测量
采用Sequenom MassARRAY平台对FAIM2启动子甲基化水平进行定量检测。采用问卷调查的方式收集了静态生活行为和运动数据。采用多重线性回归检测肥胖和消瘦儿童间不同生活行为方式对甲基化水平影响的差异。


结果
FAIM2启动子区域7个CpG位点的甲基化水平与静态生活行为显著相关。其中，位点–975、位点–413、位点–362/–360和位点–353/–349的P值分别是0.000 04、0.000 09、0.000 6和0.000 05。在运动水平小于150分钟/周的肥胖和消瘦病例间有4个CpG位点的甲基化水平存在差异。


结论
本研究首次证明，在肥胖和消瘦儿童间的FAIM2启动子甲基化水平与静态生活行为和运动的关系存在差异。本研究结果表明生活行为可能介导FAIM2参与肥胖的过程。
miR-184 mediates hyperoxia-induced injury by targeting cell death and angiogenesis signalling pathways in the developing lung
Journal: European Respiratory Journal (2020)

MicroRNAs (miRs) have been shown to disrupt normal lung development and function by interrupting alveolarization and vascularisation leading to development of bronchopulmonary dysplasia (BPD). Here we report that miR-184 has a critical role in the induction of BPD phenotype characterised by abnormal alveolarization and pulmonary angiogenesis in the developing lung. We observed an increased expression of miR-184 in BPD clinical specimens: tracheal aspirates (TA), human neonatal lungs with BPD and in fetal human lung Type II alveolar epithelial cells (TIIAECs) exposed to hyperoxia. Consistent with this, we also detected an upregulated miR-184-3p expression in whole lungs, in freshly isolated TIIAECs from lungs of hyperoxia-induced experimental BPD mice and in fetal mice lung TIIAECs exposed to hyperoxia. We demonstrate that overexpression of miR-184-3p exacerbates the BPD pulmonary phenotype, while downregulation of miR-184-3p expression ameliorated the BPD phenotype and also improved respiratory function. We identified miR-184 specific targets: platelet-derived growth factor-beta ( Pdgf- β) and friend of Gata 2 ( Fog2 ), also known as zinc finger protein family member ( Zfpm2 ), and show that they are critically involved in pulmonary alveolarization and angiogenesis. Using cell-based luciferase analysis, downregulation of miR-184-3p expression and gene knockdown of miR-184-3p targets Pdgf-β and Fog2 in lung TIIAECs and endothelial cells, we mechanistically show that inhibition of miR-184-3p expression improves pulmonary alveolarization by regulating PDGF-β/AKT/Foxo3/Bax, Bcl 2 signalling and enhances angiogenesis by Fog2/VEGF-A/Angiopoietin-1/2 pathway. Collectively, these data suggest that the use of miR-184-3p specific inhibitors may act as novel therapeutic interventions to control the adverse effects of hyperoxia on lung development and function.
脑出血合并黏液性水肿昏迷误诊一例
Authors: 卢万勤, 赵春林, 欧阳宁艳
Journal: BMJ (2005)

患者女,51岁,因言语困难伴右侧肢体活动不便15 h于2004年2月24日入院.既往有高血压病史1年余.查体:血压180/110 mm Hg(1 mm Hg=0.133 kPa),嗜睡,运动性失语,右侧肢体中枢性偏瘫.头颅CT示左基底节区脑出血,量约10 ml.入院后给予脱水、降颅压等治疗,意识逐渐清醒.但于2月27日再次出现嗜睡,且逐渐加重,并于3月1日出现意识不清,呈浅昏迷状态,血压93/64 mm Hg,呼吸12次/min,浅慢不规则,时有暂停现象。
CIFeR: A novel Clinician-lead Intervention to address Fear of cancer Recurrence (FCR) in breast cancer survivors.
Authors: Jia Liu, Kim Tam Bui, Anastasia Serafimovska, Belinda Emma Kiely, Mun Ngah Hui, Annabel Goodwin, Catriona M. McNeil, Daniel SJ Costa, Phyllis Butow, Jane McNeil Beith
Journal: Journal of Clinical Oncology (2020)

12115 Background: FCR affects 50-70% of cancer survivors. There are no validated oncologist-delivered FCR interventions. This multicentre, single-arm study sought to determine the helpfulness, feasibility and efficacy of an oncologist-delivered FCR intervention. Methods: Women were invited to participate if they had completed local treatment, chemotherapy and/or HER2 targeted therapy for early stage breast cancer and had a FCR score &gt;0 on the 42-item FCR Inventory. The brief intervention, delivered by their medical oncologist at routine follow-up, entailed 1) FCR normalisation; 2) provision of personalised prognostic information; 3) take-home education sheet on recurrence symptoms; and 4) advice on managing worry. Consultations were audio-recorded. FCR, need for help, depression and anxiety were assessed before the intervention (T0), and at one week (T1) and three months (T2) after the intervention. Satisfaction with the intervention was assessed at T1. The primary outcome was participant-rated helpfulness. Secondary outcomes included feasibility (response rate, time taken for intervention) and efficacy. Results: Five oncologists delivered the intervention to 61 women (255 women invited; response rate 24%). The mean age was 57 ± 13 years. The mean time since breast cancer diagnosis was 2.5 ± 1.3 years. Forty-three (72%) were on adjuvant hormonal therapy. Overall, 58 women (95%) found the intervention helpful and 59 (98%) would recommend it to others. FCR severity, and the proportion of women with clinically significant FCR decreased significantly over time. There were no significant changes in unmet need, depression, or anxiety. Forty (66%) of consultations were recorded. Mean consultation length was 22 minutes (range 12-37 minutes) and mean intervention length was 9 minutes (3-20 minutes). The intervention was perceived as useful and feasible by oncologists, all of whom have used components of the intervention to help manage FCR in other breast cancer patients. Conclusions: A brief oncologist-delivered intervention to address FCR is helpful and feasible, and has shown preliminary efficacy in reducing FCR. Plans for an implementation study amongst oncologists in Australia are underway. Clinical trial information: ACTRN12618001615279 . [Table: see text]
Clinical reasoning in differential diagnosis of arthritis
Authors: Xiaoyan Cai, Xiuyan Yang
Journal: BMJ (2017)

关节疼痛在临床上非常常见，疾病种类繁多，不同关节炎的治疗和预后大不相同。如何进行鉴别诊断，对于全科医生来说非常重要。本文就是否伴随晨僵、发病年龄、性别、症状、受累关节部位、关节肿胀数目、持续时间、影像学特点及实验室检查等多个方面对关节炎的鉴别诊断进行一些阐述，以提高全科医生对关节炎的鉴别能力。
Safety and outcome of allogeneic stem cell transplantation in mycosis fungoides and Sezary syndrome: A seven-year tertiary center analysis.
Authors: Selami Kocak Toprak, Guldane Cengiz Seval, Erden Atilla, Sinem Civriz Bozdag, Meltem Kurt Yuksel, Pervin Topcuoglu, Bengu Nisa Akay, Hatice Sanli, Gunhan Gurman, Muhit Ozcan
Journal: Journal of Clinical Oncology (2020)

e20038 Background: Allogeneic hematopoietic stem cell transplantation (AHSCT) is a promising strategy for treatment of advanced-stage mycosis fungoides/Sezary syndrome (MF/SS). In this study, we retrospectively analyzed the outcomes of AHSCT for MF/SS and found that MF/SS appears to be susceptible to graft versus leukemia (GVL) effects. Methods: We studied outcomes in a consecutive series of 14 patients with MM/SS who underwent AHSCT between January 2012-November 2019 at our center. Treatment response was evaluated according to the Consensus WHO criteria for MF/SS. Results: The median age of patients 51.5 years (range: 28-68 years). Eight patients had MF and six patients had SS. Nine of those 14 patients had large cell transformation. All patients had advanced disease (stages IIB: n = 1, IIIA: n = 2, IIIB: n = 6, IVA1: n = 2 and IVB = 3). The median interval from disease onset to AHSCT was 3.7 years (range: 10.5 mos-11.3 years). All of the patients received at least three lines of treatments prior to AHSCT. Four patients achieved CR (3 are alive and still in CR) and all the other patients had progressive disease (PD) at the time of AHSCT. Graft source was peripheral blood stem cell in all patients. Six patients underwent transplantation from 9/10 HLA-matched unrelated donors and eight patients from HLA-identical sibling donors. Most of the patients (12/14) received RIC regimen (Flu-Cy-TBI) and ATG-Fresenius also added the protocol in AHSCT from unrelated donors. Five patients had stage 2-4 cutaneous acute GVHD and chronic GVHD developed in four patients (mild: n = 1, moderate: n = 2, severe: n = 1). Six patients who underwent transplantation at PD attained CR after AHSCT however relapse occurred in four patients at a median of four months (range: 3-10 months) after HSCT. At the time of data collection, nine patients have deceased; three were due to early TRM, three were underlying disease, two had aspergillus infection and one had nocardia infection. Two patients were alive with disease being treated by the Brentixumab vedotin, gemcitabine and DLI. With a median follow up period of 8.7 months after AHSCT, the estimated 1-year OS and PFS was 40.4%±5% and 35.4%±3.9%, respectively. Conclusions: AHSCT is an effective treatment option for advanced-stage MF/SS. Further studies are warranted to improve the outcome after AHSCT.
Cediranib in combination with olaparib in patients without a germline BRCA1/2 mutation with recurrent platinum-resistant ovarian cancer: Phase IIb CONCERTO trial.
Authors: Jung-min Lee, Richard G. Moore, Sharad A. Ghamande, Min S. Park, John Paul Diaz, Julia A. Chapman, James Erasmus Kendrick, Brian M. Slomovitz, Krishnansu Sujata Tewari, Elizabeth S. Lowe, Tsveta Milenkova, Sanjeev Kumar, Mike Dymond, Iwanka Kozarewa, Joyce F. Liu
Journal: Journal of Clinical Oncology (2020)

6056 Background: A Phase I trial (NCT01116648) of cediranib (cedi) in combination with olaparib (ola) (cedi + ola) demonstrated an overall response rate of 44% in patients (pts) with recurrent ovarian cancer (OC), including pts without a deleterious or suspected deleterious gBRCAm (non-gBRCAm; Liu et al. Eur J Cancer 2013). The subsequent Phase II trial (NCT01116648) showed significant improvement in progression-free survival (PFS) with cedi + ola versus ola monotherapy in recurrent platinum-sensitive OC pts, notably in non-gBRCAm pts (Liu et al. Lancet Oncol 2014). We report data from the Phase IIb, single-arm, open-label CONCERTO study investigating cedi + ola in non-gBRCAm pts with recurrent platinum-resistant OC who had received ≥3 previous lines of therapy for advanced OC (NCT02889900). Methods: Pts with disease progression &lt;6 months from the last receipt of platinum-based chemotherapy received cedi tablets (30 mg once daily) plus ola tablets (200 mg twice daily) until progression or unacceptable toxicity. gBRCAm pts were ineligible. Primary endpoint: objective response rate (ORR) by independent central review (ICR; RECIST 1.1). Key secondary endpoints: PFS and safety. Results: 60 pts from the USA were included (median age: 64.5 years; median number of previous systemic treatment regimens: 4 [range: 2–9]; previous bevacizumab: 53). All pts had high-grade OC (90% serous; 3.3% clear cell; 3.3% endometrioid; 3.3% other). 7% of pts had tumor BRCA2 (confirmed somatic) mutations, 80% of pts had no tumor BRCA mutation (non-tBRCAm) and 13% of pts were not evaluable for tBRCAm. Five (8%) pts who were non-tBRCAm carried somatic homologous recombination repair gene mutations (FoundationOne Clinical Trial Assay, Foundation Medicine, Inc). The Table shows results of key endpoints. Most common grade ≥3 adverse events (AEs) that occurred in pts were hypertension (30%), fatigue (22%) and diarrhea (13%). 37% of pts reported serious AEs, of which nausea (7%) was most common. Dose interruptions, reductions and discontinuations were caused by AEs in 55%, 18% and 18% of pts, respectively, who received cedi + ola. Conclusions: Cedi + ola showed evidence of antitumor activity in heavily pretreated non-gBRCAm pts with recurrent platinum-resistant OC. Toxicity was manageable with dose modifications. Clinical trial information: NCT02889900. [Table: see text]
Key issues in primary prevention of stroke
Authors: Dan Li, Quantao Zeng, Ming Liu, Mangmang Xu
Journal: BMJ (2016)

脑卒中的一级预防是减少脑卒中疾病负担的最佳途径之一，主要通过控制一些可干预的危险因素包括高血压、糖尿病、血脂异常、房颤及心脏病、吸烟、饮酒、偏头痛等，另外首次脑卒中风险评估与预警对于患者和医务人员都是非常有帮助的。
小儿朗格汉斯细胞组织细胞增多症长期误诊一例
Authors: 战美丽, 李书芹, 刘瑞凤, 史静, 齐风芹
Journal: BMJ (2004)

朗格汉斯细胞组织细胞增多症(LCH)是一种原因不明的免疫性疾病,主要是朗格汉斯细胞细胞在某些组织中异常增生,其临床表现复杂,涉及多个器官,且无特异性,极易误诊,特别是当病变局限于某一器官时更易误诊.我院2003年5月确诊的1例误诊时间长达3年,报告如下。