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Idiopathic hypereosinophilic syndrome and disseminated intravascular coagulation 特发性嗜酸性粒细胞增多综合征和弥散性血管内凝血
Pub Date : 2019-01-01 DOI: 10.4103/CTM.CTM_3_19
M. Abdulla
Hypereosinophilic syndromes (HESs) are conditions characterized by persistent eosinophilia of 1.5 × 109/L or higher and manifestations of end‐organ involvement or dysfunction attributable to the eosinophilia. Immunological, infectious, and malignant disorders can be associated with HESs but can be even idiopathic. Disseminated intravascular coagulation (DIC) associated with HES is rare. We report a case of HES complicated by DIC which improved with treatment. A 45‐year‐old woman was admitted with generalized itching for 6 months and breathlessness for 1 week. She had a history of hemorrhoids for the past 6 years. She denied a history of weight loss, had no sick contacts, and had no history of addictions. Examination showed multiple purpuric spots over both hand and foot and a Grade 3/5 pansystolic murmur over the tricuspid area. There were no other bleeding manifestations. Hemoglobin was 10.0 g/dl, total leukocyte count – 19,700/μl (neutrophil – 39%, lymphocytes – 23%, eosinophils – 33%, and monocytes – 5%), platelet count – 57,000/L, and erythrocyte sedimentation rate – 8 mm in 1 h. Peripheral smear showed hemolysis, severe eosinophilia, and moderate thrombocytopenia [Figure 1a]. Direct and indirect Coombs tests were negative. Renal function tests, blood sugar, serum electrolytes, urinalysis, and chest radiograph were all normal. Liver function tests showed indirect hyperbilirubinemia. Prothrombin time and activated partial thromboplastin time (aPTT) were both prolonged. Serum lactate dehydrogenase, D‐dimer, and fibrin degradation product level were high. HIV, HbsAg, and HCV serologies were negative. Bone marrow examination showed trilineage maturation and myeloid hyperplasia with increase in eosinophils and its precursors [Figure 1b]. Workup for hypereosinophilia including stool examination for parasites, antinuclear antibody, and antineutrophil cytoplasmic antibody was negative. Cytogenetic studies for detecting FIP1L1‐PDGFRA rearrangement, CHIC2 deletion, FGFR1 rearrangement, and breakpoint cluster region‐Abelson murine leukemia were negative. Ultrasonography abdomen showed a hypoechoic lesion in the left lobe of the liver (48 mm × 38 mm). Contrast‐enhanced computed tomography of the abdomen showed an exophytic, hypodense lesion measuring 8.1 cm × 7 × cm 4.2 cm in the segment IV of the liver, and on postcontrast imaging, the lesion showed gradual centripetal filling in the arterial, portal, and delayed phases, which was suggestive of hepatic hemangioma. Echocardiogram showed thickened posterior mitral valve leaflet, moderate tricuspid and mitral regurgitation, severe pulmonary arterial hypertension, left ventricular apical and lateral wall fibrosis and right ventricular apical fibrosis, and dysfunction [Figure 2]. Contrast‐enhanced computed tomography thorax was normal. A diagnosis of DIC complicating idiopathic HES with cardiac involvement was made. She was treated with intravenous methylprednisolone 1 g daily for 3 days, which was followed
嗜酸性粒细胞增多综合征(HESs)是一种以嗜酸性粒细胞持续增加1.5 × 109/L或更高为特征的疾病,并表现为终末器官受累或由嗜酸性粒细胞增多引起的功能障碍。免疫,感染性和恶性疾病可与HESs相关,但甚至可以是特发性的。弥散性血管内凝血(DIC)与HES相关是罕见的。我们报告一例HES合并DIC,经治疗后病情好转。一名45岁女性因全身瘙痒6个月,呼吸困难1周入院。她有过去6年的痔疮病史。她否认自己有减肥史,没有生病的接触者,也没有吸毒史。检查显示手脚多处紫斑,三尖瓣区3/5级全收缩期杂音。无其他出血表现。血红蛋白10.0 g/dl,总白细胞计数- 19,700/μl(中性粒细胞- 39%,淋巴细胞- 23%,嗜酸性粒细胞- 33%,单核细胞- 5%),血小板计数- 57,000/L,红细胞沉降- 1小时8毫米。外周涂片显示溶血,严重嗜酸性粒细胞增多,中度血小板减少[图1a]。直接和间接Coombs试验均为阴性。肾功能、血糖、血清电解质、尿液分析、胸片检查均正常。肝功能检查显示间接高胆红素血症凝血酶原时间和活化部分凝血活酶时间(aPTT)均延长。血清乳酸脱氢酶、D -二聚体和纤维蛋白降解产物水平较高。HIV、HbsAg和HCV血清学均为阴性。骨髓检查显示三岁成熟和髓样增生,嗜酸性粒细胞及其前体增加[图1b]。嗜酸性粒细胞增多症检查包括粪便寄生虫检查、抗核抗体和抗中性粒细胞细胞质抗体均为阴性。检测FIP1L1 - PDGFRA重排、CHIC2缺失、FGFR1重排和断点簇区- Abelson小鼠白血病的细胞遗传学研究均为阴性。腹部超声示肝左叶低回声病灶(48mm × 38mm)。腹部增强计算机断层扫描显示肝脏第四节长8.1 cm × 7 cm × 4.2 cm的外生性低密度病变,增强后成像显示动脉、门静脉逐渐向心性充盈,呈延迟期,提示肝血管瘤。超声心动图显示二尖瓣后小叶增厚,中度三尖瓣及二尖瓣返流,重度肺动脉高压,左室心尖及侧壁纤维化及右室心尖纤维化,功能障碍[图2]。胸部造影增强计算机断层扫描正常。诊断DIC合并特发性HES累及心脏。患者静脉注射甲基强的松龙1 g/ d,连续3天,随后口服强的松龙(1 mg/kg体重)。治疗后患者的呼吸困难有所改善,但嗜酸性粒细胞持续存在,因此开始每天服用1 g羟基脲,嗜酸性粒细胞计数恢复正常。HESs嗜酸性粒细胞增多的病因可以是原发性(髓性)、继发性(淋巴细胞驱动)或未知的。HES可分为六种临床变型:骨髓增生性HE/HES (M‐HE/M‐HES)、淋巴细胞变型HE/HES (L‐HE/L‐HES)、重叠型HE/相关型HE/HES、家族性HE/HES和特发性HES。血栓栓塞是HES的常见并发症(25%),死亡率高(5%-10%因此死亡)。导致HES衍生凝血功能障碍的实际机制尚不清楚。嗜酸性粒细胞释放的四种主要颗粒蛋白,包括主要碱性蛋白、嗜酸性粒细胞衍生的神经毒素、嗜酸性粒细胞阳离子蛋白和嗜酸性粒细胞过氧化物酶,被认为是导致高凝状态的原因
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引用次数: 0
Progress in clinical follow-up study of dendritic cells combined with cytokine-induced killer for stomach cancer 树突状细胞联合细胞因子诱导杀手治疗胃癌的临床随访研究进展
Pub Date : 2019-01-01 DOI: 10.4103/ctm.ctm_32_19
Ling Wang, Run Wan, Cong Chen, Ruiliang Su, Yumin Li
Stomach cancer is a common malignant disease and is generally associated with mortality. Immunotherapy, including dendritic cells combined with cytokine-induced killer cells (DC-CIK), poses a significant presence. Follow-up plays a vital role in immunotherapy with stomach cancer by effectively predicting recurrence, promptly diagnosing disease, and early intervening to improve clinical outcome and survival rate. Follow-up guideline takes into these terms which are response evaluation criteria in solid tumors, health-related quality of life, tumor markers, T-lymphocyte subsets, and cytokine on the basis of the National Comprehensive Cancer Network guideline. At the same time, the high-level follow-up team is necessary. Hitherto, there is no specific follow-up guide for immunotherapy. This article reviews the follow-up of DC-CIK with stomach cancer, and it is expected that more researchers focus on this issue to draw up utility guidelines of immunotherapy for stomach cancer.
胃癌是一种常见的恶性疾病,通常与死亡率有关。免疫疗法,包括树突状细胞结合细胞因子诱导的杀伤细胞(DC-CIK),提出了重要的存在。随访能有效预测胃癌的复发,及时诊断病情,早期干预,提高临床转归和生存率,在胃癌免疫治疗中起着至关重要的作用。随访指南在国家综合癌症网络指南的基础上,纳入了实体瘤应答评价标准、健康相关生活质量、肿瘤标志物、t淋巴细胞亚群和细胞因子。同时,高水平的后续团队也是必要的。迄今为止,尚无针对免疫治疗的特异性随访指南。本文就DC-CIK治疗胃癌的随访情况进行综述,希望有更多的研究者关注这一问题,制定胃癌免疫治疗的实用指南。
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引用次数: 0
Research and development of anticancer agents under the guidance of biomarkers 生物标志物指导下抗癌药物的研究与开发
Pub Date : 2019-01-01 DOI: 10.4103/ctm.ctm_2_19
Xiaohui Xu, Guoyu Qiu, L. Ji, Ruiping Ma, Zi-long Dang, R. Jia, Bo Zhao
At present, cancer ranks first as the cause of death in the world, necessitating the need to develop new anticancer agents. As a probe, biomarkers can indicate the biological and pharmacological activity of anticancer agents and are thus valuable in predicting their effectiveness during the research and development phase. This paper reviews the research on the biomarker-guided prediction of the efficacy of anticancer agents. We infer that, in the process of the development of anticancer agents, reasonable selection of biomarkers can improve the accuracy of the development of anticancer agents.
目前,癌症是世界上第一大死亡原因,因此有必要开发新的抗癌药物。作为一种探针,生物标志物可以指示抗癌药物的生物学和药理学活性,因此在研究和开发阶段预测其有效性具有重要价值。本文综述了生物标志物引导下抗癌药物疗效预测的研究进展。我们推断,在抗癌药物的开发过程中,合理选择生物标志物可以提高抗癌药物开发的准确性。
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引用次数: 8
Inhibitory effect of hyaluronidase-4 in a rat spinal cord hemisection model 透明质酸酶-4在大鼠脊髓半切模型中的抑制作用
Pub Date : 2019-01-01 DOI: 10.4103/ctm.ctm_30_18
Xipeng Wang, Mitsuteru Yokoyama, Ping Liu
Objective: In this study, the effects of anti-hyaluronidase-4 (Hyal-4) antibody on the histological changes of Hyal-4 and the corresponding chondroitin sulfate proteoglycan (CSPG) expression in the rat spinal cord hemisection model were examined. Methods: After creating a rat spinal cord hemisection injury, experiments were conducted by administering anti-Hyal-4 antibody or control immunoglobulin G by intraspinal injection as a single dose, or intrathecal administration, using osmotic pumps, as multiple doses. Frozen sections of the injured spinal cord were made after a single-dose administration on days 1 and 4 and 1 week or at 1, 2, 3, and 4 weeks after the start of pump-aided injections. Immunofluorescence studies were then conducted using CS56 for CSPGs and anti-glial fibrillary acidic protein antibody for reactive astrocytes. Results: No difference was observed between the test and control groups in the single-dose administration of the antibody. In pump-aided administration, CSPGs in the control group decreased at 4 weeks, but those in the anti-Hyal-4 antibody administered group did not. Conclusion: Persistent suppression of Hyal-4 allowed CSPGs to remain and also increase in the rat spinal cord hemisection model, confirming Hyal-4 as an endogenous digestive enzyme of CSPGs.
目的:观察抗透明质酸酶-4 (Hyal-4)抗体对大鼠脊髓半切模型中Hyal-4的组织学变化及相应的硫酸软骨素蛋白多糖(CSPG)表达的影响。方法:在造成大鼠脊髓半切损伤后,采用单剂量脊髓内注射抗hyal -4抗体或对照免疫球蛋白G,或多剂量渗透泵鞘内注射。在泵辅助注射开始后的第1、4、1周或第1、2、3、4周单剂量给药后,制作损伤脊髓的冷冻切片。然后用CS56对CSPGs和抗胶质纤维酸性蛋白抗体对反应性星形胶质细胞进行免疫荧光研究。结果:实验组与对照组单次给药无显著差异。在泵辅助给药时,对照组的CSPGs在4周时下降,而抗hyal -4抗体给药组的CSPGs则没有下降。结论:持续抑制Hyal-4可使CSPGs在大鼠脊髓半切模型中保留并增加,证实了Hyal-4是CSPGs的内源性消化酶。
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引用次数: 1
Novel insights into the role of bacterial gut microbiota in hepatocellular carcinoma 细菌肠道菌群在肝细胞癌中的作用的新见解
Pub Date : 2018-11-01 DOI: 10.4103/ctm.ctm_8_18
Lei Zhang, Guoyu Qiu, Xiaohui Xu, Yufeng Zhou, Rui-ming Chang
Hepatocellular carcinoma (HCC) mostly develops in patients with chronic liver disease, driven by a vicious cycle of liver injury, inflammation, and regeneration. Increasing evidence points to the influence of bacterial gut microbiota influence on chronic liver disease and the development of HCC. We will review how bacterial gut microbiota contributes to HCC and relevant therapeutic strategies, focusing on alterations of the bacterial gut microbiota at different disease stages and mechanisms by which it contributes to disease progression and HCC development in different types of liver diseases.
肝细胞癌(HCC)主要发生在慢性肝病患者中,由肝损伤、炎症和再生的恶性循环驱动。越来越多的证据表明,细菌肠道微生物群对慢性肝病和HCC的发展有影响。我们将回顾细菌肠道微生物群如何促进HCC和相关的治疗策略,重点关注细菌肠道微生物群在不同疾病阶段的改变及其在不同类型肝脏疾病中促进疾病进展和HCC发展的机制。
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引用次数: 0
Central odontogenic fibroma with unusual presenting symptoms 表现不寻常的中枢性牙源性纤维瘤
Pub Date : 2018-11-01 DOI: 10.4103/ctm.ctm_29_18
Aanchal Tandon, B. Bordoloi, Safia Siddiqui, R. Jaiswal
The central odontogenic fibroma is a rare benign odontogenic neoplasm. It arises from either dental follicle or periodontal ligament or dental papilla. The lesion is characterized by collagenous fibrous connective tissue, containing varying number of islands or strands of odontogenic epithelium. Here, we present a case report of odontogenic fibroma in a 20-year-old female patient with unusual presenting symptoms.
摘要中央牙源性纤维瘤是一种罕见的良性牙源性肿瘤。它起源于牙滤泡或牙周韧带或牙乳头。病变的特征是胶原纤维结缔组织,含有不同数量的岛状或链状牙源性上皮。在此,我们报告一位20岁的女性牙源性纤维瘤患者,其临床表现异常。
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引用次数: 0
Assessing the feasibility of using deformable registration for onboard multimodality-based target localization in radiation therapy 评估可变形配准用于机载多模态放射治疗目标定位的可行性
Pub Date : 2018-11-01 DOI: 10.4103/ctm.ctm_31_18
Ge Ren, Yawei Zhang, L. Ren
Aim: This study aims to explore the feasibility of using prior images and deformable image registration to generate onboard multimodality images to improve the soft tissue contrast of cone beam computed tomography (CBCT), for target localization in radiation therapy. Methods: B-spline-based deformable registration is used to register magnetic resonance/computed tomography (MR/CT) images with CBCT images to generate synthetic onboard MR/CT images for onboard target localization. Liver, prostate, and breast patient data were used in the study to investigate the feasibility of the method. Results: Most of the tumor volume defined by the onboard synthetic images was covered by the planning target volume (PTV) based on the shifts applied in clinical practice. For 6 liver cases, 5 prostate cases, and all the breast cases, the synthetic images allowed the reduction of the PTV margin to 1.5–7 mm, 1–4 mm, and 1–1.5 mm, respectively. The dose to the normal tissue can be reduced based on the optimized margin. Conclusion: This study demonstrated the feasibility of using onboard synthetic multimodality imaging to improve the soft tissue contrast for target localization in low contrast regions. This new technique holds great promises to optimize the PTV margin and improve the treatment accuracy in radiation therapy.
目的:探讨利用先验图像和可变形图像配准生成机载多模态图像的可行性,以提高锥形束计算机断层扫描(CBCT)的软组织对比度,为放射治疗中的靶标定位提供依据。方法:采用基于b样条的可变形配准方法,将磁共振/CT (MR/CT)图像与CBCT图像进行配准,生成合成的机载MR/CT图像,用于机载目标定位。本研究使用肝脏、前列腺和乳房患者的资料来探讨该方法的可行性。结果:根据临床应用的移位,大部分由机载合成图像定义的肿瘤体积被规划靶体积(PTV)覆盖。肝脏6例,前列腺5例,乳腺全部,合成图像使PTV切缘分别缩小到1.5 ~ 7mm, 1 ~ 4mm, 1 ~ 1.5 mm。对正常组织的剂量可在优化裕度的基础上减小。结论:本研究证明了利用机载合成多模态成像提高低对比度区域软组织对比度定位的可行性。这项新技术在优化PTV边界和提高放射治疗的治疗准确性方面具有很大的前景。
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引用次数: 0
Research advancement in the tumor biomarker of hepatocellular carcinoma 肝细胞癌肿瘤标志物的研究进展
Pub Date : 2018-11-01 DOI: 10.4103/ctm.ctm_32_18
Qingfu Du, Xiaoying Ji, Guang-Jin Yin, D. Wei, Pengcheng Lin, Yong-jun Lu, Yu-guang Li, Qiaohong Yang, Shizhu Liu, Jinliang Ku, Wen-Hua Guan, Yuanzhi Lu
Hepatocellular carcinoma (HCC) is the third most common cause of mortality due to malignancy next to gastric cancer and esophageal cancer. Several causal factors have been proposed to be involved in the pathogenesis of HCC including regional and age difference, hepatitis virus, aflatoxin, chemicals, liver cirrhosis, and family heredity. Compared to the initial symptoms of HCC, the late symptoms are more obvious, which are liver pain, fatigue, emaciation, jaundice, and ascites. This paper summarized 30 biomarkers from organs, tissues, cells, and subcellular systems to be used for the diagnosis, detection, staging, and evaluation of the HCC so as to predict or prognoses diseases. The biomarker degrees of healthy people have a certain range of indicators. Pathological and clinical diagnostic methods are mainly used to detect various biomarkers related to HCC, such as the common detection of alpha-fetoprotein to detect HCC. Diverse indicators are determined by the modern technology so that we can explore and clarify the possible indicators associated with the pathogenesis of diseases in the organisms. In general, if one or more biomarkers are beyond the normal range, it would predict the presence of HCC in the body. Here, we try to provide a significant contribution toward clinical screening, prediction, diagnosis, treatment, and follow-up monitoring of HCC and related diseases through elucidating the conception, production and influencing factors, sources, and biochemical indicator-associated tumor markers.
肝细胞癌(HCC)是仅次于胃癌和食管癌的第三大常见死亡原因。肝癌发生的原因包括地区和年龄差异、肝炎病毒、黄曲霉毒素、化学物质、肝硬化和家族遗传等。与HCC的初期症状相比,晚期症状更为明显,表现为肝脏疼痛、乏力、消瘦、黄疸、腹水等。本文总结了30种来自器官、组织、细胞和亚细胞系统的生物标志物,可用于HCC的诊断、检测、分期和评估,从而预测或预后。健康人的生物标志物程度有一定的指标范围。病理和临床诊断方法主要用于检测与HCC相关的各种生物标志物,如常用的检测甲胎蛋白来检测HCC。现代技术确定了多种指标,使我们能够探索和阐明与生物体疾病发病机制相关的可能指标。一般来说,如果一个或多个生物标志物超出正常范围,就可以预测体内存在HCC。在此,我们试图通过阐明HCC及相关疾病的概念、产生及影响因素、来源、生化指标相关肿瘤标志物,为HCC及相关疾病的临床筛查、预测、诊断、治疗及随访监测做出重要贡献。
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引用次数: 1
Challenges and advances in the management of pediatric intracranial germ cell tumors: A case report and literature review 儿科颅内生殖细胞肿瘤治疗的挑战与进展:1例报告及文献复习
Pub Date : 2018-09-01 DOI: 10.4103/ctm.ctm_36_17
G. Millen, Karen A Manias, A. Peet, J. Adamski
Intracranial germ cell tumors are a heterogeneous group of tumors, broadly classified into germinomatous, nongerminomatous, or teratoma subtypes. Treatment has evolved over recent decades to include multimodal therapy combining surgery, radiotherapy, and chemotherapy. Although the majority of intracranial germs cell tumors are treated successfully, management can be fraught with complexities and present significant clinical challenges. Bifocal disease is well described, but rare, and therefore its behavior is not well characterized, particularly in nongerminomatous disease. This case report presents an interesting case, with both rare and common complications, in particular, to emphasize the challenges of germ cell tumor management. With a focus on bifocal disease, we review the published cases and highlight how advanced imaging and magnetic resonance spectroscopy can be used in management. Advances in biology, targeted agents, and novel diagnostic tools are also discussed.
颅内生殖细胞肿瘤是一类异质性的肿瘤,大致分为生殖细胞瘤、非生殖细胞瘤和畸胎瘤亚型。近几十年来,治疗已经发展到包括手术、放疗和化疗相结合的多模式治疗。虽然大多数颅内细菌细胞瘤治疗成功,但管理可能充满复杂性,并呈现显着的临床挑战。双局部疾病已被很好地描述,但很罕见,因此其行为没有很好地表征,特别是在非包瘤性疾病中。本病例报告提出了一个有趣的病例,罕见和常见的并发症,特别强调生殖细胞肿瘤管理的挑战。以双焦点疾病为重点,我们回顾了已发表的病例,并强调了先进的成像和磁共振波谱在治疗中的应用。在生物学,靶向药物和新的诊断工具的进展也进行了讨论。
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引用次数: 2
IDH gene mutation in glioma 胶质瘤中IDH基因突变
Pub Date : 2018-09-01 DOI: 10.4103/ctm.ctm_27_18
Le-ping Liu, Xue-Jun Li
Glioma is one of the most common intracranial malignant tumors. Its development is associated with mutations in the isocitrate dehydrogenase (IDH) gene. IDH plays an important role in the tricarboxylic acid cycle, and when mutated, it can downregulate the expression of α-ketoglutarate and convert it to 2-hydroxypentaric acid (2-HG), activating the HIF-1 pathway to promote the development of glioma. This article briefly describes the role of IDH mutations in glioma development and progression and its relationship with other gene mutations. This information may provide a new perspective toward the treatment, molecular pathological grading, and prognosis of glioma.
胶质瘤是最常见的颅内恶性肿瘤之一。它的发展与异柠檬酸脱氢酶(IDH)基因的突变有关。IDH在三羧酸循环中发挥重要作用,突变后可下调α-酮戊二酸的表达,将其转化为2-羟基戊二酸(2-HG),激活HIF-1通路,促进胶质瘤的发生发展。本文简要介绍IDH突变在胶质瘤发生发展中的作用及其与其他基因突变的关系。这些信息可能为胶质瘤的治疗、分子病理分级和预后提供新的视角。
{"title":"IDH gene mutation in glioma","authors":"Le-ping Liu, Xue-Jun Li","doi":"10.4103/ctm.ctm_27_18","DOIUrl":"https://doi.org/10.4103/ctm.ctm_27_18","url":null,"abstract":"Glioma is one of the most common intracranial malignant tumors. Its development is associated with mutations in the isocitrate dehydrogenase (IDH) gene. IDH plays an important role in the tricarboxylic acid cycle, and when mutated, it can downregulate the expression of α-ketoglutarate and convert it to 2-hydroxypentaric acid (2-HG), activating the HIF-1 pathway to promote the development of glioma. This article briefly describes the role of IDH mutations in glioma development and progression and its relationship with other gene mutations. This information may provide a new perspective toward the treatment, molecular pathological grading, and prognosis of glioma.","PeriodicalId":9428,"journal":{"name":"Cancer Translational Medicine","volume":"45 1","pages":"129 - 133"},"PeriodicalIF":0.0,"publicationDate":"2018-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74262518","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
期刊
Cancer Translational Medicine
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