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The efficacy and safety of second salvage autologous transplantation in myeloma patients 骨髓瘤患者第二次挽救性自体移植的疗效和安全性
Pub Date : 2024-07-16 DOI: 10.3389/pore.2024.1611851
R. Bicsko, Renáta Nyilas, Róbert Szász, L. Váróczy, Attila Kiss, Miklós Udvardy, Árpád Illés, L. Gergely
Despite the availability of many novel therapies for multiple myeloma, it remains an incurable disease with relapse fated in almost all patients. In the era of modern agents, second autologous stem cell transplantation still holds its role in patients relapsing after first-line autologous transplant. The authors reviewed a single-center experience with a second auto-SCT for relapsed multiple myeloma. Thirty patients had received a salvage auto-SCT at the institution. The median follow-up after diagnosis was 86 months, and the median time between transplants was 59.1 months. Response before second ASCT was the following: CR – 11 cases, VGPR – 9 cases, PR – 10 cases. Most patients received reduced dose (140 mg/m2) of melphalan as a conditioning regimen for the second auto-SCT. Treatment-related mortality was 3%. With a median follow-up time of 34 months after the second transplant, median progression-free survival was 24 months. The median PFS in the patients achieving CR or VGPR at day 100 after the second transplantation was 32 months. By 15 months, all patients achieved only partial remission progressed, with a median PFS of 8.5 months. During the follow-up period, no MDS or AML developed, and the frequency of second malignancy was also low, 3%. In conclusion, second autologous stem cell transplantation is a well-tolerated and effective treatment option for relapsed multiple myeloma in selected patients, though with a shorter PFS than in first remission.
尽管多发性骨髓瘤有了许多新型疗法,但它仍然是一种无法治愈的疾病,几乎所有患者都会复发。在现代药物时代,对于一线自体干细胞移植后复发的患者,二次自体干细胞移植仍有其作用。作者回顾了单中心第二次自体干细胞移植治疗复发多发性骨髓瘤的经验。该机构有30名患者接受了挽救性自体移植。确诊后的中位随访时间为86个月,两次移植之间的中位间隔时间为59.1个月。第二次自体移植前的反应情况如下:CR - 11例,VGPR - 9例,PR - 10例。大多数患者在第二次自体造血干细胞移植中接受了减量(140 毫克/平方米)美啡仑作为条件治疗方案。治疗相关死亡率为 3%。第二次移植后的中位随访时间为34个月,中位无进展生存期为24个月。第二次移植后第100天达到CR或VGPR的患者的中位无进展生存期为32个月。到15个月时,所有仅获得部分缓解的患者都出现了进展,中位无进展生存期为8.5个月。在随访期间,没有出现MDS或AML,二次恶性肿瘤的发生率也很低,仅为3%。总之,对于复发的多发性骨髓瘤患者,二次自体干细胞移植是一种耐受性良好且有效的治疗选择,尽管PFS比首次缓解期短。
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引用次数: 0
The efficacy outcomes in non-small cell lung cancer patients treated with PD axis inhibitor agents - a population-based study of the Vojvodina region 使用 PD 轴抑制剂治疗非小细胞肺癌患者的疗效结果 - Vojvodina 地区的一项人口研究
Pub Date : 2024-07-12 DOI: 10.3389/pore.2024.1611717
Nensi Lalić, M. Bojović, D. Bursác, D. Bokan, Vesna Ćeriman Krstić, Ivan Kuhajda, Biljana Parapid, Sanja Tomić, Aleksandar Šipka
Background: By 2021, the FDA approved the use of the drugs pembrolizumab and atezolizumab in the first-line treatment of patients with high positivity of programmed death ligand-1 (PD-L1) in locally advanced and metastatic non-small-cell-lung cancer (NSCLC). This approval was the result of statistically significant evidence from international, multicentric clinical studies that all reported increasing progression-free survival (PFS) and overall survival (OS) in these patients.Methods: In our study, we reported the demographic and clinical characteristics of 79 patients diagnosed with NSCLC with expression of PD-L1 ≥50% from January 2019 to December 2022 at the Institute for Pulmonary Diseases of Vojvodina, who received pembrolizumab therapy as the first-line treatment. Patients were divided according to the histological type of lung cancer as adenocarcinoma (ADC) or squamous cell carcinoma (SCC) of the lung. In 52 of the 79 patients, PFS and in 32 of them overall survival (censored OS) was shown according to the histological type of tumor, the tumor proportion score (TPS) of PDL-1 expression, and the metastatic status within the Tumor Nodes Metastasis (TNM) disease classification. Independent factors of death outcome were shown by multivariable proportional hazard regression analysis.Results: The study included 79 patients diagnosed with NSCLC with an expression of PD-L1 ≥50%, 50 (63.3%) patients with ADC, and 29 (36.7%) patients with SCC, whose 55 (69.6%) PDL-1 expression was obtained from broncho biopsy (BB). The majority of patients, 49 (62%), had a TPS PD-L1 score of 51%–79%. Median, PFS for adenocarcinoma was 22 months and censored OS was 27 months, while for squamous cell carcinoma, median PFS was 12 months, and censored OS was 21 months. M1b disease stage, which was the most common in patients, had a PFS of 16 months and a censored OS of 18 months.Conclusion: Pembrolizumab monotherapy in patients with NSCLC in the fourth stage of the disease and with the positivity of the immune checkpoint protein TPS PD-L1 above 50% represents a safe therapy that allows a satisfactory period without disease progression and overall survival with acceptable treatment complications.
背景:到 2021 年,FDA 批准使用药物 pembrolizumab 和 atezolizumab 一线治疗程序性死亡配体-1(PD-L1)高度阳性的局部晚期和转移性非小细胞肺癌(NSCLC)患者。这项批准是国际多中心临床研究统计证据的结果,这些研究均报告这些患者的无进展生存期(PFS)和总生存期(OS)有所延长:在我们的研究中,我们报告了 Vojvodina 肺病研究所在 2019 年 1 月至 2022 年 12 月期间确诊的 79 例 PD-L1 表达≥50%、接受 pembrolizumab 治疗作为一线治疗的 NSCLC 患者的人口统计学和临床特征。患者根据肺癌组织学类型分为肺腺癌(ADC)和肺鳞癌(SCC)。根据肿瘤组织学类型、PDL-1表达的肿瘤比例评分(TPS)以及肿瘤结节转移(TNM)疾病分类中的转移状态,对79名患者中的52名患者的PFS和32名患者的总生存期(经删减的OS)进行了分析。多变量比例危险回归分析显示了死亡结果的独立因素:研究纳入了79例PD-L1表达≥50%的NSCLC患者、50例(63.3%)ADC患者和29例(36.7%)SCC患者,其中55例(69.6%)患者的PDL-1表达是通过支气管活检(BB)获得的。大多数患者(49人(62%))的TPS PD-L1评分为51%-79%。腺癌的中位生存期为22个月,删减后的生存期为27个月,而鳞癌的中位生存期为12个月,删减后的生存期为21个月。患者中最常见的M1b疾病分期的PFS为16个月,OS为18个月:结论:Pembrolizumab单药治疗处于疾病第四阶段且免疫检查点蛋白TPS PD-L1阳性率超过50%的NSCLC患者是一种安全的治疗方法,可在无疾病进展和总生存期内获得满意的疗效,且治疗并发症可接受。
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引用次数: 0
From prenatal diagnosis to surgical treatment: two case reports of congenital granular cell epulis 从产前诊断到手术治疗:两例先天性颗粒细胞外皮瘤报告
Pub Date : 2024-07-10 DOI: 10.3389/pore.2024.1611834
Yibing Han, Wen Qiu, Yu Zhang, Mengmeng Hua, Shaohua Liu, Zuoqing Dong
Herein, we detail a multidisciplinary approach and sequential treatment for two infants with congenital granular cell epulis (CGCE). Ultrasonic examinations at 34 weeks of gestation revealed prominent oral masses in both fetuses. To devise a carefully considered treatment strategy, a comprehensive multidisciplinary consultation including oral and maxillofacial surgeons, pediatricians, obstetricians, and anesthesiologists was convened. Following cesarean sections, the lesions were successfully removed, measuring approximately 30 × 15 mm and 30 × 20 mm in size, respectively. Immunohistochemical analysis showed that vimentin was positive, S-100 protein was negative, and NSE protein and CD68 protein were negative. These findings underscore the importance of prenatal diagnosis of congenital granular cell epulis for the effective management of these rare benign conditions.
在此,我们详细介绍了对两名患有先天性颗粒细胞性赘生物(CGCE)的婴儿采取的多学科方法和连续治疗。妊娠34周时的超声波检查发现,两个胎儿的口腔均有明显肿块。为了制定一个经过深思熟虑的治疗策略,他们召集了一次全面的多学科会诊,包括口腔颌面外科医生、儿科医生、产科医生和麻醉师。剖腹产手术后,病灶被成功切除,大小分别约为 30 × 15 毫米和 30 × 20 毫米。免疫组化分析显示,波形蛋白呈阳性,S-100 蛋白呈阴性,NSE 蛋白和 CD68 蛋白呈阴性。这些发现强调了产前诊断先天性颗粒细胞性赘生物对有效治疗这类罕见良性病症的重要性。
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引用次数: 0
Flow cytometry in the differential diagnosis of myelodysplastic neoplasm with low blasts and cytopenia of other causes 流式细胞术在骨髓增生异常肿瘤伴低血小板和其他原因引起的全血细胞减少症的鉴别诊断中的应用
Pub Date : 2024-07-08 DOI: 10.3389/pore.2024.1611811
M. Plander, Mária Kányási, Tamás Szendrei, J. Skrapits, B. Timár
Myelodysplastic neoplasms (MDS) are characterized by cytopenia, morphologic dysplasia, and genetic abnormalities. Multiparameter flow cytometry (FCM) is recommended in the diagnostic work-up of suspected MDS, but alone is not sufficient to establish the diagnosis. Our aim was to investigate the diagnostic power of FCM in a heterogeneous population of patients with cytopenia, excluding cases with increased blast count.We analyzed bone marrow samples from 179 patients with cytopenia (58 MDS, 121 non-MDS) using a standardized 8-color FCM method. We evaluated the sensitivity, specificity, and accuracy of several simple diagnostic approaches, including Ogata score, extended Ogata score, the WHO and ELN iMDSFlow recommended “3 aberrations in two cell compartments method,” and the combination of the Ogata score and “3 aberrations in two cell compartments method.” The patients were followed until the diagnosis was confirmed, with a median follow-up of 2 months (range 0.2–27).The combination of Ogata score and “3 aberrations in two cell compartments method” achieved the highest diagnostic accuracy (78%) with sensitivity and specificity 61% and 86%, respectively. When using only the “3 aberrations in two cell compartments method,” the accuracy was 77% with a sensitivity of 72% and a specificity of 79%. The most frequently observed etiologies among the false positive cases were substrate deficiencies, inflammation/infection, or toxic effects. MDS can be excluded in all these cases after a thorough clinical evaluation and a relatively short follow-up.FCM remains an important but supplementary part in an integrated diagnostic process of MDS with low blasts. The combination of the Ogata score and the “3 aberrations in two cell compartments method” slightly improves accuracy compared to the detection of “3 aberrations in two cell compartments method” alone.
骨髓增生异常性肿瘤(MDS)以全血细胞减少、形态发育不良和基因异常为特征。多参数流式细胞术(FCM)被推荐用于疑似骨髓增生异常性肿瘤的诊断工作,但它本身并不足以确定诊断。我们采用标准化的 8 色 FCM 方法分析了 179 例全血细胞减少患者(58 例 MDS,121 例非 MDS)的骨髓样本。我们评估了几种简单诊断方法的敏感性、特异性和准确性,包括绪方评分、扩展绪方评分、WHO 和 ELN iMDSFlow 推荐的 "两个细胞区 3 个畸变法",以及绪方评分和 "两个细胞区 3 个畸变法 "的组合。对患者进行随访直至确诊,中位随访时间为 2 个月(0.2-27 个月)。绪方评分和 "两个细胞区 3 个畸变法 "的组合诊断准确率最高(78%),敏感性和特异性分别为 61% 和 86%。如果仅使用 "两个细胞区 3 个畸变法",准确率为 77%,敏感性为 72%,特异性为 79%。在假阳性病例中,最常见的病因是基质缺乏、炎症/感染或毒性作用。经过全面的临床评估和相对较短的随访,所有这些病例均可排除 MDS。绪方评分与 "两个细胞区 3 个畸变法 "相结合,与单独检测 "两个细胞区 3 个畸变法 "相比,准确性略有提高。
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引用次数: 0
The roles of phosphorylation of signaling proteins in the prognosis of acute myeloid leukemia 信号蛋白磷酸化在急性髓性白血病预后中的作用
Pub Date : 2024-07-05 DOI: 10.3389/pore.2024.1611747
Adrienn Márton, Katalin Beáta Veres, F. Erdődi, Miklós Udvardy, Árpád Illés, László Rejtő
Signaling pathways of Retinoblastoma (Rb) protein, Akt-kinase, and Erk-kinase (extracellular signal-regulated kinase) have an important role in the pathogenesis of acute myeloid leukemia. Constitutive activation of these proteins by phosphorylation contributes to cell survival by regulation of cell cycle, proliferation and proapoptotic signaling processes. According to previous data phosphorylated forms of these proteins represent a worse outcome for cancer patients. We investigated the presence of phosphorylated Rb (P-Rb), Akt (P-Akt) and Erk (P-Erk) proteins by Western blot technique using phospho-specific antibodies in bone marrow or peripheral blood samples of 69 AML patients, 36 patients with myelodysplastic syndrome (MDS) and 10 healthy volunteers. Expression level of PTEN (Phosphatase and tensin homolog) and PHLPP (PH domain and leucine-rich repeat Protein Phosphatase) phosphatases, the negative regulators of Akt kinase pathway were also examined. We tested the effect of these proteins on survival and on the correlation with known prognostic features in AML. We found 46.3% of AML patients had detectable P-Rb, 34.7% had P-Akt and 28.9% had P-Erk protein. 66.1% of patients expressing PTEN, 38.9% PHLPP, 37.2% both PTEN and PHLPP and 32.2% neither PTEN nor PHLPP phosphatases. Compared to nucleophosmin mutation (NPMc) negative samples P-Erk was significantly less in nucleophosmin mutated patients, P-Rb was significantly less in patients’ group with more than 30 G/L peripheral leukocyte count by diagnosis. PHLPP was significantly present in FAB type M5. The expression of P-Rb represented significant better overall survival (OS), while P-Akt represented significantly worse event-free survival (EFS) in unfavorable cytogenetics patients. The presence of both PHLPP and PTEN phosphatases contributes to better OS and EFS, although the differences were not statistically significant. We confirmed significant positive correlation between P-Akt and PHLPP. Assessing the phosphorylation of Rb, Akt and Erk may define a subgroup of AML patients who would benefit especially from new targeted treatment options complemented the standard chemotherapy, and it may contribute to monitoring remission, relapse or progression of AML.
视网膜母细胞瘤(Rb)蛋白、Akt 激酶和 Erk 激酶(细胞外信号调节激酶)的信号通路在急性髓性白血病的发病机制中起着重要作用。这些蛋白通过磷酸化被持续激活,通过调节细胞周期、增殖和促凋亡信号转导过程促进细胞存活。根据以往的数据,这些蛋白的磷酸化形式意味着癌症患者的预后较差。我们使用磷酸化特异性抗体,通过 Western 印迹技术检测了 69 名 AML 患者、36 名骨髓增生异常综合征(MDS)患者和 10 名健康志愿者骨髓或外周血样本中磷酸化 Rb(P-Rb)、Akt(P-Akt)和 Erk(P-Erk)蛋白的存在情况。我们还检测了 Akt 激酶通路的负调控因子 PTEN(磷酸酶和天丝同源物)和 PHLPP(PH 结构域和富亮氨酸重复蛋白磷酸酶)磷酸酶的表达水平。我们检测了这些蛋白对存活率的影响以及与急性髓细胞性白血病已知预后特征的相关性。我们发现46.3%的急性髓细胞性白血病患者检测到P-Rb蛋白,34.7%检测到P-Akt蛋白,28.9%检测到P-Erk蛋白。66.1%的患者表达PTEN,38.9%表达PHLPP,37.2%同时表达PTEN和PHLPP,32.2%既不表达PTEN也不表达PHLPP磷酸酶。与核嗜酸基因突变(NPMc)阴性样本相比,核嗜酸基因突变患者中的P-Erk明显较少,P-Rb在诊断时外周血白细胞计数超过30 G/L的患者组中明显较少。PHLPP在FAB M5型中明显存在。在不利细胞遗传学患者中,P-Rb的表达明显提高了总生存率(OS),而P-Akt的表达则明显降低了无事件生存率(EFS)。PHLPP和PTEN磷酸酶的存在有助于提高OS和EFS,尽管差异在统计学上并不显著。我们证实,P-Akt与PHLPP之间存在明显的正相关性。对Rb、Akt和Erk的磷酸化进行评估,可以确定急性髓细胞性白血病患者的一个亚组,这些患者将特别受益于新的靶向治疗方案和标准化疗的补充,而且还有助于监测急性髓细胞性白血病的缓解、复发或进展。
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引用次数: 0
The role of immunotherapy in early-stage and metastatic NSCLC 免疫疗法在早期和转移性非小细胞肺癌中的作用
Pub Date : 2024-07-04 DOI: 10.3389/pore.2024.1611713
Attila Lieber, Attila Makai, Zsuzsanna Orosz, Tamás Kardos, Susil Joe Isaac, Ilona Tornyi, Nóra Bittner
In the past decade we have seen new advances and thus remarkable progress in the therapeutic options for non-small cell lung cancer (NSCLC). Among cytostatic therapies with new approaches in molecularly targeted therapies, we see new developments in a wide range of applications for immunotherapies. In this review we discuss the new potential modalities for the use of immune checkpoint inhibitors (ICIs) in the frontlines, including in early-stage (perioperative) and metastatic settings. The perioperative use of ICIs in both neoadjuvant and adjuvant settings may show benefits for patients. In early-stage NSCLC (from stage IIB and above) a multimodality approach is recommended as the gold standard for the treatment. After surgical resection platinum-based adjuvant chemotherapy has been the standard of care for many years. Based on the benefit of disease-free survival, the approval of adjuvant atezolizumab and adjuvant pembrolizumab was a significant breakthrough. In the metastatic setting, the use of immune checkpoint inhibitors with chemotherapy, regardless of PD-L1 expression or ICI alone (PD-L1 expression equal to or greater than 50%) also improves overall survival and progression-free survival.
在过去的十年中,我们看到了非小细胞肺癌(NSCLC)治疗方案的新进展,从而取得了显著进步。在细胞抑制疗法和分子靶向疗法的新方法中,我们看到了免疫疗法广泛应用的新进展。在这篇综述中,我们将讨论免疫检查点抑制剂(ICIs)在前线(包括早期(围手术期)和转移期)使用的新的潜在模式。在新辅助治疗和辅助治疗中,围手术期使用 ICIs 可为患者带来益处。对于早期 NSCLC(IIB 期及以上)患者,建议将多模式疗法作为治疗的金标准。多年来,手术切除后的铂类辅助化疗一直是治疗的标准。基于无病生存期的优势,辅助治疗阿特珠单抗和辅助治疗彭博利珠单抗的批准是一项重大突破。在转移性环境中,免疫检查点抑制剂与化疗联合使用,不论PD-L1表达或单用ICI(PD-L1表达等于或大于50%),也能改善总生存期和无进展生存期。
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引用次数: 0
Decreasing incidence and mortality of lung cancer in Hungary between 2011 and 2021 revealed by robust estimates reconciling multiple data sources 通过协调多种数据来源的可靠估算,揭示了 2011 年至 2021 年匈牙利肺癌发病率和死亡率的下降趋势
Pub Date : 2024-06-03 DOI: 10.3389/pore.2024.1611754
Gabriella Gálffy, Géza Tamás Szabó, L. Tamási, Veronika Müller, J. Moldvay, V. Sárosi, A. Kerpel-Fronius, Tamás Kardos, E. Csada, Z. Pápai-Székely, Zoltán Szász, Z. Király, Gábor Hódi, Zsuzsanna Kovács, Éva Balogh, K. Kovács, Miklós Darida, Viktória Buga, G. Rokszin, Z. Abonyi-Tóth, Zoltán Kiss, Z. Vokó, K. Bogos
Hungary has repeatedly been shown to have the highest cancer-related mortality and incidence in Europe. Despite lung cancer being the most abundant malignant diagnosis in Hungary, numerous concerns have been raised recently regarding the bias inherent to reported incidence estimates. Re-analysis of reimbursement claims has been suggested previously by our group as an alternative approach, offering revised figures of lung cancer incidence between 2011 and 2016. Leveraging on this methodology, we aimed at updating Hungarian lung cancer incidence estimates with an additional 5 years (2017–2021), including years affected by the COVID-19 pandemic. Additionally, we also attempted to improve the robustness of estimates by taking additional characteristics of the patient pathway into account.Lung cancer patients between 2011 and 2021 were identified based on reimbursement-associated ICD-10 codes, histology codes and time patterns. Multiple query architectures were tested for sensitivity and compared to official estimates of the Hungarian National Cancer Registry (HNCR). Epidemiological trends were estimated by Poisson-regression, corrected for age and sex.A total of 89,948 lung cancer patients diagnosed in Hungary between 2011 and 2021 have been identified by our study. In 2019 alone, 7,887 patients were diagnosed according to our optimized query. ESP2013 standardized rate was estimated between 92.5/100,000 (2011) and 78.4/100,000 (2019). In 2019, standardized incidence was 106.8/100,000 for men and 59.7/100,000 for women. Up until the COVID-19 pandemic, lung cancer incidence was decreasing by 3.18% (2.1%–4.3%) yearly in men, while there was no significant decrease in women. Young age groups (40–49 and 50–59) featured the largest improvement, but women aged 60–79 are at an increasing risk for developing lung cancer. The COVID-19 pandemic resulted in a statistically significant decrease in lung cancer incidence, especially in the 50–59 age group (both sexes).Our results show that using an optimized approach, re-analysis of reimbursement claims yields robust estimates of lung cancer incidence. According to this approach, the incidence rate of male lung cancer is declining in Hungary, in concordance with the trend observed for lung cancer mortality. Among women aged 60–79, the incidence of lung cancer has risen, requiring more attention in the near future.
匈牙利多次被证明是欧洲癌症死亡率和发病率最高的国家。尽管肺癌是匈牙利发病率最高的恶性肿瘤,但最近人们对报告的发病率估计值的内在偏差提出了许多担忧。此前,我们小组曾建议将重新分析报销单据作为一种替代方法,提供 2011 年至 2016 年肺癌发病率的修订数据。利用这一方法,我们旨在更新匈牙利肺癌发病率估计值,增加了 5 年(2017-2021 年),包括受 COVID-19 大流行影响的年份。此外,我们还试图通过考虑患者路径的其他特征来提高估计值的稳健性。2011 年至 2021 年期间的肺癌患者是根据与报销相关的 ICD-10 编码、组织学编码和时间模式确定的。测试了多种查询架构的灵敏度,并与匈牙利国家癌症登记处(HNCR)的官方估计值进行了比较。通过泊松回归估算了流行病学趋势,并对年龄和性别进行了校正。我们的研究共确定了 2011 年至 2021 年期间在匈牙利确诊的 89948 名肺癌患者。根据我们的优化查询,仅2019年就有7887名患者被确诊。ESP2013的标准化发病率估计在92.5/100,000(2011年)和78.4/100,000(2019年)之间。2019年,男性的标准化发病率为106.8/100,000,女性为59.7/100,000。在 COVID-19 大流行之前,男性肺癌发病率每年下降 3.18%(2.1%-4.3%),而女性则没有显著下降。年轻年龄组(40-49 岁和 50-59 岁)的改善幅度最大,但 60-79 岁的女性罹患肺癌的风险越来越高。COVID-19大流行导致肺癌发病率出现了统计学意义上的显著下降,尤其是在50-59岁年龄组(男女均有)。根据这种方法,匈牙利男性肺癌发病率正在下降,与肺癌死亡率的趋势一致。在 60-79 岁的妇女中,肺癌发病率有所上升,需要在不久的将来给予更多关注。
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引用次数: 0
Case Report: Effective management of adalimumab-induced acquired hemophilia A with the CyDRI protocol 病例报告:使用 CyDRI 方案有效治疗阿达木单抗引起的获得性 A 型血友病
Pub Date : 2024-05-23 DOI: 10.3389/pore.2024.1611720
Andrea Ceglédi, Árpád Bátai, János Dolgos, Mónika Fekete, László Gopcsa, Viktória Király, Gergely Lakatos, György Nagy, Zsuzsanna Szemlaky, Andrea Várkonyi, Beáta Vilimi, Gábor Mikala, Imre Bodó
Acquired Hemophilia A (AHA) is a rare autoimmune disorder characterized by the emergence of inhibitors that specifically target coagulation Factor VIII, frequently resulting in severe bleeding episodes.We conducted a retrospective analysis of the medical records of a 68-year-old male patient who presented with adalimumab-induced AHA.The patient received adalimumab, a tumor necrosis factor inhibitor antibody, as part of his treatment for rheumatoid arthritis. The patient’s clinical journey, characterized by intense bleeding and coagulopathy, was effectively managed with the application of recombinant Factor VIIa (rFVIIa) and the CyDRi protocol.The case emphasizes the importance of prompt coagulation assessment in patients with bleeding symptoms receiving disease-modifying therapy for rheumatoid arthritis that includes adalimumab therapy, considering the rare yet life-threatening nature of AHA. Additionally, this report provides an extensive review of the existing literature on drug-induced AHA, with a special emphasis on cases linked to immunomodulatory medications. Through this two-pronged approach, our report aims to enhance understanding and awareness of this severe complication among healthcare providers, promoting timely diagnosis and intervention.
获得性血友病A(AHA)是一种罕见的自身免疫性疾病,其特点是出现专门针对凝血因子VIII的抑制剂,经常导致严重的出血发作。我们对一名68岁男性患者的病历进行了回顾性分析,该患者因阿达木单抗诱发AHA而接受了阿达木单抗(一种肿瘤坏死因子抑制剂抗体)治疗,作为类风湿性关节炎治疗的一部分。该病例强调了在接受包括阿达木单抗在内的类风湿性关节炎疾病调节疗法时,考虑到 AHA 的罕见性和生命危险,对有出血症状的患者及时进行凝血功能评估的重要性。此外,本报告还广泛综述了有关药物诱发 AHA 的现有文献,并特别强调了与免疫调节药物有关的病例。通过这种双管齐下的方法,我们的报告旨在加强医护人员对这种严重并发症的理解和认识,促进及时诊断和干预。
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引用次数: 0
Novel diagnostic processes and challenges in bronchoscopy 支气管镜检查的新诊断程序和挑战
Pub Date : 2024-05-21 DOI: 10.3389/pore.2024.1611774
Z. Pápai-Székely, Gábor Grmela, Veronika Sárosi
Diagnostic bronchoscopy is a minimally invasive procedure that plays a crucial role in the diagnosis and management of various respiratory conditions. This paper explores the advancements in technology that have revolutionized the field and focuses on the new diagnostic procedures in bronchoscopy that have emerged in recent years. These innovative techniques have expanded the diagnostic capabilities of bronchoscopy, allowing for more accurate and comprehensive evaluation of respiratory conditions. This paper will also discuss the challenges in the diagnostic process with bronchoscope.
诊断性支气管镜检查是一种微创手术,在诊断和治疗各种呼吸系统疾病中发挥着至关重要的作用。本文探讨了使这一领域发生革命性变化的技术进步,并重点介绍了近年来出现的新型支气管镜诊断程序。这些创新技术拓展了支气管镜的诊断能力,使呼吸系统疾病的评估更加准确和全面。本文还将讨论支气管镜诊断过程中面临的挑战。
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引用次数: 0
Can long-read sequencing tackle the barriers, which the next-generation could not? A review 长读数测序能否解决新一代测序无法解决的障碍?综述
Pub Date : 2024-05-16 DOI: 10.3389/pore.2024.1611676
Nikolett Szakállas, Barbara K. Barták, Gábor Valcz, Z. Nagy, István Takács, Béla Molnár
The large-scale heterogeneity of genetic diseases necessitated the deeper examination of nucleotide sequence alterations enhancing the discovery of new targeted drug attack points. The appearance of new sequencing techniques was essential to get more interpretable genomic data. In contrast to the previous short-reads, longer lengths can provide a better insight into the potential health threatening genetic abnormalities. Long-reads offer more accurate variant identification and genome assembly methods, indicating advances in nucleotide deflect-related studies. In this review, we introduce the historical background of sequencing technologies and show their benefits and limits, as well. Furthermore, we highlight the differences between short- and long-read approaches, including their unique advances and difficulties in methodologies and evaluation. Additionally, we provide a detailed description of the corresponding bioinformatics and the current applications.
遗传疾病的大规模异质性要求对核苷酸序列的改变进行更深入的研究,以发现新的靶向药物攻击点。新测序技术的出现对于获得更多可解释的基因组数据至关重要。与以往的短读数相比,长读数能更好地洞察潜在的威胁健康的基因异常。长读数可提供更准确的变异识别和基因组组装方法,表明核苷酸偏转相关研究取得了进展。在这篇综述中,我们介绍了测序技术的历史背景,并说明了它们的优势和局限性。此外,我们还强调了短读方法和长读方法之间的差异,包括它们在方法和评估方面的独特进展和困难。此外,我们还详细介绍了相应的生物信息学和当前的应用。
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引用次数: 0
期刊
Pathology and Oncology Research
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