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Ocular Adnexal Lymphoma: Epidemiology and Clinical Characteristics 眼附件淋巴瘤:流行病学和临床特征
Q3 Medicine Pub Date : 2022-04-30 DOI: 10.4999/uhod.225970
A. Sarici
The aim of the study is to emphasize the frequency, clinical presentation, histopathologic features and TNM staging for each type of ocular adnexal lymphoma (OAL), and investigating treatment results and prognosis in our region. A retrospective review of 54 patients treated for primary and secondary OAL between Jan 2012 and Jan 2019 was made. Epidemiologic data, clinical characteristics of the tumor and recurrence free survival rates were evaluated. Patients with ocular adnexal lymphoma included 27 (50%) women and 27 (50%) men, with a mean age of 60.38±15.36 (range: 18-93) years at the time of presentation. Mean follow-up time was 40.88±20.75 (range 1-84) months. Histopathological diagnosis was extranodal marginal zone lymphoma in 75.9%, diffuse large B-cell lymphoma in 14.8%, chronic lymphocytic leukemia/small lymphocytic lymphoma in 5.6%, mantle cell lymphoma in 1.9% and follicular cell lymphoma in 1.9% of patients. Among 54 patients with OAL 66.7% had orbital, 22.2% had conjunctival, 5.6% had orbital plus conjunctival, 3.7% had orbital plus conjunctival and choroidal, 1.9% had conjunctival plus choroidal involvement. No recurrences were observed in 87.1% of patients during their follow-up. Our data indicates patient epidemiologic presentation of a single center.
本研究的目的是强调各类型眼附件淋巴瘤(OAL)的发病频率、临床表现、组织病理学特征和TNM分期,并探讨本地区的治疗效果和预后。回顾性分析了2012年1月至2019年1月期间接受原发性和继发性OAL治疗的54例患者。评估流行病学资料、肿瘤临床特征及无复发生存率。眼附件淋巴瘤患者中女性27例(50%),男性27例(50%),平均年龄为60.38±15.36(范围:18-93)岁。平均随访时间40.88±20.75(1 ~ 84)个月。组织病理学诊断为结外边缘区淋巴瘤75.9%,弥漫性大b细胞淋巴瘤14.8%,慢性淋巴细胞白血病/小淋巴细胞淋巴瘤5.6%,套细胞淋巴瘤1.9%,滤泡细胞淋巴瘤1.9%。54例OAL患者中,66.7%为眼眶受累,22.2%为结膜受累,5.6%为眼眶+结膜受累,3.7%为眼眶+结膜+脉络膜受累,1.9%为结膜+脉络膜受累。87.1%的患者在随访期间无复发。我们的数据表明患者的流行病学表现为单一中心。
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引用次数: 0
Calreticulin Mutations in Philadelphia Chromosome Negative Myeloproliferative Neoplasms 费城染色体阴性骨髓增生性肿瘤中的钙网蛋白突变
Q3 Medicine Pub Date : 2022-04-30 DOI: 10.4999/uhod.225925
G. Gulbay
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引用次数: 0
The Effect of Metformin on SIK1 and SIK2 in MCF-7 Cell as an Anticancer Agent 二甲双胍对MCF-7细胞SIK1和SIK2的抗癌作用
Q3 Medicine Pub Date : 2022-04-30 DOI: 10.4999/uhod.226162
Eser Çakmak
Recent studies have shown that the use of metformin prevents the development and spread of cancer. Metformin may show this effect by increasing SIK1 and SIK2 gene expression. For this purpose, MCF-7 cells cultured in appropriate media were divided into 8 groups (1) control, (2) 10 ng/mL TGF- β 1, (3) 1.25 mM Metformin, (4) 2.5mM Metformin, (5) 20mM Metformin, (6) 1.25 mM Metform-in+10 ng/ml TGF- β 1, (7) 2.5mM Metformin+10 ng/ml TGF- β 1 and (8) 20mM Metformin+10 ng/ml TGF- β 1 doses were administered, respectively. PCR was performed for SIK1 and SIK2 genes, with GAPDH being the reference gene. Application of 10 ng/ml TGF- β 1 to MCF-7 cell significantly increased expression level of SIK1 mRNA by 1.6 fold. In non-invasive (TGF- β 1 not administered) MCF-7 cell, 2.5 mM and 20 mM metformin increased expression levels of SIK1 mRNA by 1.8, 3.4 fold and SIK2 mRNA by 1.6 and 3.3 fold respectively. In invasive (TGF- β 1 administered) MCF-7 cell, 1.25, 2.5 and 20 mM metformin increased expression levels of SIK1 mRNA by 3.5, 3.7, 4 fold; and SIK2 mRNA by 1.9, 2.4, 3.5 fold, respectively. Metformin increased SIK1 and SIK2 gene expression dose-dependently in non-invasive and invasive MCF-7 cells, more significantly in invasive ones. The increase in the SIK1 gene was greater than in SIK2. In the light of these results, investigating the effects of metformin on SIK1 and SIK2 genes in different TGF- β 1 sensitive cancer types may open new doors for cancer treatment.
最近的研究表明,使用二甲双胍可以防止癌症的发展和扩散。二甲双胍可能通过增加SIK1和SIK2基因表达来显示这种作用。为此,在适当的培养基中培养的MCF-7细胞分为8组(1)对照组,(2)10 ng/mL TGF- β 1, (3) 1.25 mM二甲双胍,(4)2.5mM二甲双胍,(5)20mM二甲双胍,(6)1.25 mM二甲双胍+10 ng/mL TGF- β 1, (7) 2.5mM二甲双胍+10 ng/mL TGF- β 1和(8)20mM二甲双胍+10 ng/mL TGF- β 1。以GAPDH为内参基因,对SIK1和SIK2基因进行PCR检测。10 ng/ml TGF- β 1作用于MCF-7细胞后,SIK1 mRNA的表达水平显著提高1.6倍。在非侵袭性(未给药TGF- β 1) MCF-7细胞中,2.5 mM和20 mM二甲双胍分别使SIK1 mRNA表达量增加1.8倍、3.4倍,使SIK2 mRNA表达量增加1.6倍和3.3倍。在侵袭性(TGF- β 1给药)MCF-7细胞中,1.25、2.5和20 mM二甲双胍分别使SIK1 mRNA的表达水平提高3.5倍、3.7倍和4倍;SIK2 mRNA分别增加1.9倍、2.4倍、3.5倍。二甲双胍增加了非侵袭性和侵袭性MCF-7细胞中SIK1和SIK2基因的表达,并呈剂量依赖性,在侵袭性MCF-7细胞中更为显著。SIK1基因的表达量大于SIK2。根据这些结果,研究二甲双胍对不同TGF- β 1敏感癌症类型中SIK1和SIK2基因的影响可能为癌症治疗打开新的大门。
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引用次数: 0
A Patient with Monoclonal Gammopathy of Undetermined Significance and Detected Philadelphia Chromosome 1例意义不明的单克隆γ病并检测到费城染色体
Q3 Medicine Pub Date : 2022-04-06 DOI: 10.14302/issn.2372-6601.jhor-22-4133
T. Tran, Jennifer Cai, P. Ji, Xin Qing
BackgroundMonoclonal gammopathy of undetermined significance (MGUS) and chronic myeloid leukemia (CML) are diseases of different lineages. The diagnosis of both MGUS and CML in the same patient is a rare occurrence and has not been reported in much literature.Case PresentationWe describe a 56-year-old man with a history of rheumatoid arthritis incidentally found to have an increase in IgA paraprotein. With less than 10% monoclonal plasma cells on the bone marrow biopsy and absence of hypercalcemia, renal failure, anemia and bone lesions, MGUS was diagnosed. The conventional cytogenetics at the time showed the presence of the Philadelphia chromosome in 30% of metaphases. However, there was no morphologic evidence of CML in the peripheral blood or bone marrow. Patient received no treatment and lost follow-up until 3 years later when a routine CBC showed leukocytosis and thrombocytosis. CML, chronic phase was diagnosed following a bone marrow aspiration and biopsy with Philadelphia chromosome observed in 100% of metaphases. Patient was treated with imatinib and later switched to dasatinib and complete molecular remission was continued to be achieved.Discussion and ConclusionHere we report a case of pre-leukemic CML as an incidental finding during the diagnosis of MGUS. The possible underlying mechanisms of the association are discussed although the exact cause of the coexistence is unclear.
背景:未确定意义单克隆γ病(MGUS)和慢性髓系白血病(CML)是不同谱系的疾病。在同一患者中同时诊断MGUS和CML是一种罕见的情况,在很多文献中没有报道。我们描述了一个56岁的男性与类风湿关节炎的历史偶然发现有增加的IgA副蛋白。骨髓活检中单克隆浆细胞少于10%,且无高钙血症、肾功能衰竭、贫血和骨骼病变,诊断为MGUS。当时的常规细胞遗传学显示费城染色体存在于30%的中期。然而,在外周血或骨髓中没有CML的形态学证据。患者未接受任何治疗并失去随访,直到3年后常规CBC显示白细胞增多和血小板增多。慢性粒细胞白血病,慢性期诊断后骨髓穿刺和活检,费城染色体在中期观察到100%。患者先用伊马替尼治疗,后来改用达沙替尼,并继续获得完全的分子缓解。讨论与结论我们在此报告一例白血病前CML是在MGUS诊断过程中偶然发现的。尽管共存的确切原因尚不清楚,但仍讨论了这种关联的可能潜在机制。
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引用次数: 0
Association between Perceived Stress and Neutropenia in Patients with Leukemia under Chemotherapy. 白血病化疗患者感知压力与中性粒细胞减少的关系。
Q3 Medicine Pub Date : 2022-04-01 DOI: 10.18502/ijhoscr.v16i2.9203
Mohsen Esfandbod, Maryam Abazaria Tehrani, Maryam Haghshomar, Pantea Arya, Bahareh Shateri Amiri, Gholamreza Toogeh, Manouchehr Keyhani

Background: The most prominent part of the cellular response of the immune system is driven by neutrophils. These cells tend to decline following chemotherapy in patients with leukemia. Neutropenia is an influential factor in the prognosis of cancer patients. Stress reduces white blood cells (WBCs) and neutrophils are linked to an increased risk of infectious diseases after chemotherapy. We investigated the association between neutropenia and perceived stress following chemotherapy. Materials and Methods: We performed a cross-sectional study on 60 patients with leukemia in a university hospital. Participants completed self-report measures, including the demographic data and perceived stress scale (PSS) questionnaire. We compared rates of neutropenia, as a measure of chemotherapy prognosis, 10 days after chemotherapy in different stress levels. Moreover, the number of patients with polymorphonuclear (PMN) under 1000/microliter was compared at different stress levels.    Results: We found that neutropenia is directly correlated with negative stress perception and inversely correlated with positive stress perception. These effects appear more prominent in patients with PMN under 1000/microliter as the number of these patients was significantly more in groups with higher negative stress and less in groups with higher positive stress scores. Conclusion: It can be concluded that stress is correlated with neutropenia, and stress management in patients with leukemia will be accompanied by better recovery outcomes and reduced risk of infectious disease.

背景:免疫系统细胞反应中最突出的部分是由中性粒细胞驱动的。白血病患者化疗后,这些细胞趋于减少。中性粒细胞减少是影响肿瘤患者预后的一个因素。压力降低白细胞和中性粒细胞与化疗后感染性疾病的风险增加有关。我们研究了化疗后中性粒细胞减少和感知压力之间的关系。材料与方法:我们对某大学医院的60例白血病患者进行了横断面研究。参与者完成自我报告测量,包括人口统计数据和感知压力量表(PSS)问卷。我们比较了在不同应激水平下化疗后10天中性粒细胞减少率,作为化疗预后的衡量指标。并比较不同应激水平下多形核(PMN)低于1000/微升的患者数量。结果:中性粒细胞减少与负性应激知觉直接相关,与正性应激知觉负相关。这些影响在PMN低于1000/微升的患者中表现得更为突出,负应激评分高的患者数量明显较多,正应激评分高的患者数量明显较少。结论:应激与中性粒细胞减少有关,对白血病患者进行应激管理可使其康复效果更好,降低感染性疾病的发生风险。
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引用次数: 0
SARS-CoV-2 Infection: A Possible Risk Factor for Incidence and Recurrence of Cancers. SARS-CoV-2感染:癌症发生和复发的可能危险因素
Q3 Medicine Pub Date : 2022-04-01 DOI: 10.18502/ijhoscr.v16i2.9205
Abdollah Jafarzadeh, Rohit Gosain, Seyed Mohammad Javad Mortazavi, Maryam Nemati, Sara Jafarzadeh, Abbas Ghaderi

COVID-19 and malignancy can affect the susceptibility of one another. Clinically recovered COVID-19 individuals display immune abnormalities that persist several months after discharge. The lymphopenia-related immunosuppression, functional exhaustion of cytotoxic lymphocytes (such as CD8+ cytotoxic T-cells and natural killer cells), hyperinflammatory responses, oxidative stress, downregulation of interferon response, development of the myeloid-derived suppressor cells, downregulation of tumor suppressor proteins and perhaps reactivation of the latent oncogenic viruses may directly and/or indirectly play a role in the cancer development and recurrence in severe COVID-19 patients. SARS-CoV-2-infected malignant patients may be at higher risk of death of their cancer than SARS-CoV-2-uninfected patients with the same cancers. On the other side, the patients with some types of cancers may be more vulnerable to SARS-CoV-2 infection compared with the non-cancerous individuals, due to their immunocompromised state resulted from malignancy, chemotherapy, and other concomitant abnormalities as well as perhaps greater expression of angiotensin-converting enzyme 2. SARS-CoV-2-infected cancerous patients are unable to produce an effective anti-virus immune response and may exhibit more severe forms of COVID-19. This review described the possible impacts of SARS-CoV-2 infection on cancer development and recurrence, and the potential cancer impacts on COVID-19 development, while the possible interventions are highlighted.

COVID-19和恶性肿瘤可相互影响易感性。临床恢复的COVID-19个体在出院后持续数月表现出免疫异常。淋巴细胞减少相关的免疫抑制、细胞毒性淋巴细胞(如CD8+细胞毒性t细胞和自然杀伤细胞)的功能衰竭、高炎症反应、氧化应激、干扰素反应的下调、髓源性抑制细胞的发育、肿瘤抑制蛋白的下调和潜在的致癌病毒的再激活可能直接和/或间接地在COVID-19重症患者的癌症发展和复发中发挥作用。感染sars - cov -2的恶性患者因癌症死亡的风险可能高于未感染sars - cov -2的相同癌症患者。另一方面,与非癌症患者相比,某些类型的癌症患者可能更容易受到SARS-CoV-2感染,这是由于恶性肿瘤、化疗和其他伴随异常导致的免疫功能低下状态,以及血管紧张素转换酶2的表达可能更高。感染sars - cov -2的癌症患者无法产生有效的抗病毒免疫反应,并可能表现出更严重的COVID-19形式。本文综述了SARS-CoV-2感染对癌症发展和复发的可能影响,以及癌症对COVID-19发展的潜在影响,并重点介绍了可能的干预措施。
{"title":"SARS-CoV-2 Infection: A Possible Risk Factor for Incidence and Recurrence of Cancers.","authors":"Abdollah Jafarzadeh,&nbsp;Rohit Gosain,&nbsp;Seyed Mohammad Javad Mortazavi,&nbsp;Maryam Nemati,&nbsp;Sara Jafarzadeh,&nbsp;Abbas Ghaderi","doi":"10.18502/ijhoscr.v16i2.9205","DOIUrl":"https://doi.org/10.18502/ijhoscr.v16i2.9205","url":null,"abstract":"<p><p>COVID-19 and malignancy can affect the susceptibility of one another. Clinically recovered COVID-19 individuals display immune abnormalities that persist several months after discharge. The lymphopenia-related immunosuppression, functional exhaustion of cytotoxic lymphocytes (such as CD8<sup>+</sup> cytotoxic T-cells and natural killer cells), hyperinflammatory responses, oxidative stress, downregulation of interferon response, development of the myeloid-derived suppressor cells, downregulation of tumor suppressor proteins and perhaps reactivation of the latent oncogenic viruses may directly and/or indirectly play a role in the cancer development and recurrence in severe COVID-19 patients. SARS-CoV-2-infected malignant patients may be at higher risk of death of their cancer than SARS-CoV-2-uninfected patients with the same cancers. On the other side, the patients with some types of cancers may be more vulnerable to SARS-CoV-2 infection compared with the non-cancerous individuals, due to their immunocompromised state resulted from malignancy, chemotherapy, and other concomitant abnormalities as well as perhaps greater expression of angiotensin-converting enzyme 2. SARS-CoV-2-infected cancerous patients are unable to produce an effective anti-virus immune response and may exhibit more severe forms of COVID-19. This review described the possible impacts of SARS-CoV-2 infection on cancer development and recurrence, and the potential cancer impacts on COVID-19 development, while the possible interventions are highlighted.</p>","PeriodicalId":38991,"journal":{"name":"International Journal of Hematology-Oncology and Stem Cell Research","volume":"16 2","pages":"117-127"},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/c9/41/IJHOSCR-16-117.PMC9547773.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40653740","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 4
Incidence and Prognostic Impact of WT-1 Gene Exon7 and 9 Mutations in Acute Promyelocytic Leukemia. WT-1基因外显子7和9突变在急性早幼粒细胞白血病中的发生率及对预后的影响
Q3 Medicine Pub Date : 2022-04-01 DOI: 10.18502/ijhoscr.v16i2.9199
Fatemeh Nejatifar, Shahrbano Rostami, Barham Chahardouli, Amir Kasaeian, Mohammad Vaezi, Hossein Kamranzadeh, Seied Asadollah Mousavi, Abolfazl Farbod, Kamran Alimoghaddam, Ardeshir Ghavamzadeh

Background: Wilms' tumor suppressor gene 1 (WT1) gene mutation has been reported to be a prognostic factor in normal-cytogenetic acute myeloid leukemia (AML) patients. Higher rates of mutation in the WT1 gene have been reported in several tumors including normal-cytogenetic AML patients. Data regarding WT1 mutations in acute promyelocytic leukemia (APL) is very scarce. In this study, we evaluated the incidence and impact of WT1 mutation on the outcome of APL patients. Materials and Methods: A total of 92 patients diagnosed with APL were studied in three distinct groups: early mortality, relapsed, and persistent complete remission. Genomic DNA of bone marrow samples of patients was analyzed. For quantification of expression levels of the WT1 gene, real-time quantitative PCR (rqPCR) was performed by a real-time PCR system. WT1 mutation and its impact on prognosis were considered the primary endpoint of the study. Statistical analysis was performed with STATA. Results: WT1 mutation frequency was 6.25% in the early mortality group (1/16 patients), 13.16% in the relapse group (5/38 patients), and 7.89% in the persistent complete remission group (3/38 patients). 8 mutations were in exon 7 and one mutation in exon 9. WT1 mutation in the relapse group was associated with a trend toward worse disease-free survival (DFS) while overall survival (OS) was not affected by WT1 mutation in univariate analysis. Patients with no mutations in WT1 and FLT3/ITD had better overall survival and disease-free survival compared to patients with mutations in the WT1 gene or FLT3/ITD in the relapse group. Conclusion: The frequency of WT1 gene mutations does not differ significantly between patients with early mortality, relapse, and persistent complete remission. The presence of WT1 mutation is associated with higher relapse and lower survival rates in relapse group patients.

背景:Wilms肿瘤抑制基因1 (WT1)基因突变已被报道为正常细胞遗传的急性髓性白血病(AML)患者的预后因素。据报道,在包括正常细胞遗传的AML患者在内的几种肿瘤中,WT1基因突变率较高。关于WT1突变在急性早幼粒细胞白血病(APL)中的数据非常少。在本研究中,我们评估了WT1突变对APL患者预后的发生率和影响。材料和方法:共研究了92例APL患者,分为三组:早期死亡、复发和持续完全缓解。分析患者骨髓样本的基因组DNA。采用实时荧光定量PCR (real-time quantitative PCR, rqPCR)检测WT1基因的表达水平。WT1突变及其对预后的影响被认为是研究的主要终点。采用STATA进行统计分析。结果:WT1突变频率在早期死亡组为6.25%(1/16),复发组为13.16%(5/38),持续完全缓解组为7.89%(3/38)。外显子7有8个突变,外显子9有1个突变。在单变量分析中,复发组的WT1突变与更差的无病生存(DFS)趋势相关,而总生存(OS)不受WT1突变的影响。与复发组中WT1基因或FLT3/ITD突变的患者相比,WT1和FLT3/ITD无突变的患者有更好的总生存期和无病生存期。结论:WT1基因突变的频率在早期死亡、复发和持续完全缓解的患者之间没有显著差异。在复发组患者中,WT1突变的存在与较高的复发率和较低的生存率相关。
{"title":"Incidence and Prognostic Impact of WT-1 Gene Exon7 and 9 Mutations in Acute Promyelocytic Leukemia.","authors":"Fatemeh Nejatifar,&nbsp;Shahrbano Rostami,&nbsp;Barham Chahardouli,&nbsp;Amir Kasaeian,&nbsp;Mohammad Vaezi,&nbsp;Hossein Kamranzadeh,&nbsp;Seied Asadollah Mousavi,&nbsp;Abolfazl Farbod,&nbsp;Kamran Alimoghaddam,&nbsp;Ardeshir Ghavamzadeh","doi":"10.18502/ijhoscr.v16i2.9199","DOIUrl":"https://doi.org/10.18502/ijhoscr.v16i2.9199","url":null,"abstract":"<p><p><b>Background:</b> Wilms' tumor suppressor gene 1 (WT1) gene mutation has been reported to be a prognostic factor in normal-cytogenetic acute myeloid leukemia (AML) patients. Higher rates of mutation in the WT1 gene have been reported in several tumors including normal-cytogenetic AML patients. Data regarding WT1 mutations in acute promyelocytic leukemia (APL) is very scarce. In this study, we evaluated the incidence and impact of WT1 mutation on the outcome of APL patients. <b>Materials and Methods</b>: A total of 92 patients diagnosed with APL were studied in three distinct groups: early mortality, relapsed, and persistent complete remission. Genomic DNA of bone marrow samples of patients was analyzed. For quantification of expression levels of the WT1 gene, real-time quantitative PCR (rqPCR) was performed by a real-time PCR system. WT1 mutation and its impact on prognosis were considered the primary endpoint of the study. Statistical analysis was performed with STATA. <b>Results:</b> WT1 mutation frequency was 6.25% in the early mortality group (1/16 patients), 13.16% in the relapse group (5/38 patients), and 7.89% in the persistent complete remission group (3/38 patients). 8 mutations were in exon 7 and one mutation in exon 9. WT1 mutation in the relapse group was associated with a trend toward worse disease-free survival (DFS) while overall survival (OS) was not affected by WT1 mutation in univariate analysis. Patients with no mutations in WT1 and FLT3/ITD had better overall survival and disease-free survival compared to patients with mutations in the WT1 gene or FLT3/ITD in the relapse group. <b>Conclusion:</b> The frequency of WT1 gene mutations does not differ significantly between patients with early mortality, relapse, and persistent complete remission. The presence of WT1 mutation is associated with higher relapse and lower survival rates in relapse group patients.</p>","PeriodicalId":38991,"journal":{"name":"International Journal of Hematology-Oncology and Stem Cell Research","volume":"16 2","pages":"74-80"},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/66/f4/IJHOSCR-16-74.PMC9547776.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40653741","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Clinical and Pathological Features of Double-Hit and Triple-Hit High-Grade B-Cell Lymphomas: A Retrospective Study from Three Portuguese Tertiary Centers. 双重打击和三重打击高级别b细胞淋巴瘤的临床和病理特征:来自三个葡萄牙三级中心的回顾性研究。
Q3 Medicine Pub Date : 2022-04-01 DOI: 10.18502/ijhoscr.v16i2.9202
Rui Almeida, Carlos Abrantes, Davide Gigliano, Rui Caetano Oliveira, Paulo Teixeira, Marta Viegas, Ângelo Rodrigues, Maria José Julião

Background: High-grade B-cell lymphoma (HGBL) with rearrangements of MYC and BCL2 and/or BCL6, called double and triple-hit lymphomas (DTH-HGBL), are lymphoid malignancies with inferior outcomes when treated with standard chemotherapy. The identification of DTH-HGBL cases is challenging, considering their variable clinical, morphologic, and immunohistochemical features. Materials and Methods: Retrospective revision of medical data of patients diagnosed with DTH-HGBL confirmed by FISH, between January 2010 and January 2020, in three Tertiary Portuguese Hospitals (Coimbra Hospital and University Center, Portuguese Oncology Institute - Coimbra and Portuguese Oncology Institute - Porto). Pathological features, morphology, and immunohistochemical profile were evaluated by at least two experienced pathologists in hematopoietic and lymphoid neoplasms. Results: The cohort included 24 patients: 33.3% triple-hit, 58.3%, MYC/BCL2 double-hit and 8.3% MYC/BCL6 double-hit. There was no gender predominance, with a median age of 62.5±14.3y, 33.3% were diagnosed as nodal disease, and 66.7% as extranodal. Morphologic features of DLBCL were present in 50% of cases, morphological features of both DLBCL and Burkitt lymphoma (DLBCL/BL) in 45.8% and 4.2% of blastoid morphology. Immunohistochemical evaluation, regarding the Hans algorithm, revealed a Germinal center (GC)/GC-like subtype in 83.3% of cases and a non-GC/non-GC-like subtype in 16.7%.  MYC was positive in 42.9% and the median proliferative index was 80±12.4%. Conclusion: DTH-HGBL has a very broad range of features. We consider that a cost-effective approach would be to perform cytogenetic analysis in DLBCL and DLBCL/BL cases with GC/GC-like subtype. MYC and BCL2 immunohistochemistry can be useful to identify patients who may benefit from more aggressive therapies, but not as tools for case selection for FISH.

背景:MYC和BCL2和/或BCL6重排的高级别b细胞淋巴瘤(HGBL),称为双重和三击淋巴瘤(DTH-HGBL),是标准化疗治疗后预后较差的淋巴样恶性肿瘤。考虑到DTH-HGBL病例的不同临床、形态学和免疫组织化学特征,鉴定DTH-HGBL病例具有挑战性。材料与方法:回顾性修订2010年1月至2020年1月葡萄牙三所三级医院(科英布拉医院和大学中心、科英布拉葡萄牙肿瘤研究所和波尔图葡萄牙肿瘤研究所)经FISH确诊的DTH-HGBL患者的医疗资料。病理特征、形态和免疫组织化学特征由至少两名经验丰富的造血和淋巴肿瘤病理学家评估。结果:该队列共纳入24例患者:三发型占33.3%,MYC/BCL2双发型占58.3%,MYC/BCL6双发型占8.3%。无性别优势,中位年龄为62.5±14.3y,诊断为结外病变占33.3%,结外病变占66.7%。50%的病例存在DLBCL的形态学特征,45.8%的病例存在DLBCL和Burkitt淋巴瘤(DLBCL/BL)的形态学特征,4.2%的病例存在囊胚形态特征。Hans算法的免疫组织化学评估显示,83.3%的病例为生发中心(GC)/GC样亚型,16.7%的病例为非GC/非GC样亚型。MYC阳性占42.9%,中位增殖指数为80±12.4%。结论:DTH-HGBL具有非常广泛的特征。我们认为对DLBCL和具有GC/GC样亚型的DLBCL/BL病例进行细胞遗传学分析是一种经济有效的方法。MYC和BCL2免疫组织化学可用于识别可能受益于更积极治疗的患者,但不能作为FISH病例选择的工具。
{"title":"Clinical and Pathological Features of Double-Hit and Triple-Hit High-Grade B-Cell Lymphomas: A Retrospective Study from Three Portuguese Tertiary Centers.","authors":"Rui Almeida,&nbsp;Carlos Abrantes,&nbsp;Davide Gigliano,&nbsp;Rui Caetano Oliveira,&nbsp;Paulo Teixeira,&nbsp;Marta Viegas,&nbsp;Ângelo Rodrigues,&nbsp;Maria José Julião","doi":"10.18502/ijhoscr.v16i2.9202","DOIUrl":"https://doi.org/10.18502/ijhoscr.v16i2.9202","url":null,"abstract":"<p><p><b>Background:</b> High-grade B-cell lymphoma (HGBL) with rearrangements of <i>MYC</i> and <i>BCL2</i> and/or <i>BCL6,</i> called double and triple-hit lymphomas (DTH-HGBL), are lymphoid malignancies with inferior outcomes when treated with standard chemotherapy. The identification of DTH-HGBL cases is challenging, considering their variable clinical, morphologic, and immunohistochemical features. <b>Materials and Methods</b>: Retrospective revision of medical data of patients diagnosed with DTH-HGBL confirmed by FISH, between January 2010 and January 2020, in three Tertiary Portuguese Hospitals (Coimbra Hospital and University Center, Portuguese Oncology Institute - Coimbra and Portuguese Oncology Institute - Porto). Pathological features, morphology, and immunohistochemical profile were evaluated by at least two experienced pathologists in hematopoietic and lymphoid neoplasms. <b>Results</b>: The cohort included 24 patients: 33.3% triple-hit, 58.3%, <i>MYC/BCL2</i> double-hit and 8.3% <i>MYC/BCL6</i> double-hit<i>.</i> There was no gender predominance, with a median age of 62.5±14.3y, 33.3% were diagnosed as nodal disease, and 66.7% as extranodal. Morphologic features of DLBCL were present in 50% of cases, morphological features of both DLBCL and Burkitt lymphoma (DLBCL/BL) in 45.8% and 4.2% of blastoid morphology. Immunohistochemical evaluation, regarding the Hans algorithm, revealed a Germinal center (GC)/GC-like subtype in 83.3% of cases and a non-GC/non-<i>GC</i>-like subtype in 16.7%.  MYC was positive in 42.9% and the median proliferative index was 80±12.4%. <b>Conclusion: </b>DTH-HGBL has a very broad range of features. We consider that a cost-effective approach would be to perform cytogenetic analysis in DLBCL and DLBCL/BL cases with GC/GC-like subtype. MYC and BCL2 immunohistochemistry can be useful to identify patients who may benefit from more aggressive therapies, but not as tools for case selection for FISH.</p>","PeriodicalId":38991,"journal":{"name":"International Journal of Hematology-Oncology and Stem Cell Research","volume":"16 2","pages":"94-102"},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/62/04/IJHOSCR-16-94.PMC9547779.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40653276","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
An Unusual Case of Hemolytic Anemia Reversed with Liver Transplantation. 肝移植逆转溶血性贫血1例。
Q3 Medicine Pub Date : 2022-04-01 DOI: 10.18502/ijhoscr.v16i2.9206
Vatsala Katiyar, Apaar Dadlani, Ishaan Vohra, Kamila Cisak, Ashutosh Barve

Spur cell anemia is acquired hemolytic anemia seen in patients with advanced liver disease, particularly in the setting of alcoholism, and warrants urgent liver transplant evaluation. We describe the case of a 58-year-old female with alcoholic cirrhosis who presented with worsening liver disease, profound anemia poorly responsive to blood transfusions, and multiple spur cells on the peripheral smear. She underwent a liver transplant, which led to the resolution of hematologic abnormalities and the need for transfusions. Our case highlights the significance of spur cell anemia as a harbinger of poor prognosis in patients with advanced liver disease and its reversibility with liver transplantation.

骨刺细胞贫血是一种获得性溶血性贫血,见于晚期肝病患者,特别是酒精中毒患者,需要紧急肝移植评估。我们描述了一例58岁的酒精性肝硬化女性,她表现为肝病恶化,严重贫血,对输血反应差,外周涂片上有多个刺细胞。她接受了肝脏移植,这导致血液异常的解决和输血的需要。我们的病例强调了骨刺细胞贫血作为晚期肝病患者预后不良的先兆的重要性,以及它在肝移植中的可逆性。
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引用次数: 0
Pediatric Cancer Registry at MAHAK Pediatric Cancer Treatment and Research Center: A Single-Center Study from Iran. MAHAK儿童癌症治疗和研究中心的儿童癌症登记:来自伊朗的单中心研究。
Q3 Medicine Pub Date : 2022-04-01 DOI: 10.18502/ijhoscr.v16i2.9201
Mohammad Faranoush, Narjes Mehrvar, Yasaman Sadeghi, Maryam Tashvighi, Mardawig Alebouyeh, Azim Mehrvar
Background: The childhood cancer registry in Iran is a hospital-based system and there is not any unique and national registry system for pediatric malignancies in Iran. According to the limitations and requirements, this study was designed to clarify the aspect of childhood malignancies in Iran and promote establishing the Iranian national childhood cancer registry system. Materials and Methods: This cross-sectional longitudinal study was implied on 1500 patients younger than 20-years old diagnosed with any malignancy and admitted at MAHAK Pediatric Cancer Treatment and Research Center (MPCTRC) from 2007 to 2014. Data collection was based on a validated questionnaire with three categories including demographic data, clinical data and type of malignancy, and outcomes. Collected data were analyzed using methods for qualitative and quantitative variables (P < 0.05) by SPSS software version 22. The survival rate was calculated by the Kaplan-Meyer method. Results: This study was implied on 1500 children with a mean age of 6.1 years old. The most common malignancy was acute leukemia (30.7%) followed by central nervous system tumors (27%). At the onset of starting treatment, the rate of conferring with relapse, metastasis, and secondary malignancies was 29%, 19.5%, and 1% respectively. In addition, 52 patients had bone marrow transplantation of whom, 14 cases died. Totally, 42% of patients died and the 3-years, 5-years, and 10-years overall survival rates were 67.7% ± 0.01, 60.3% ± 0.01, and 53.8% ± 0.01, respectively. Conclusion: Establishing a population-based pediatric cancer registry in Iran is necessary and will be useful for improving the survival rate of mentioned patients.
背景:伊朗的儿童癌症登记是一个以医院为基础的系统,在伊朗没有任何独特的和国家的儿科恶性肿瘤登记系统。根据局限性和要求,本研究旨在明确伊朗儿童恶性肿瘤方面,促进伊朗国家儿童癌症登记系统的建立。材料与方法:本横断面纵向研究纳入了2007年至2014年在MAHAK儿科癌症治疗与研究中心(MPCTRC)就诊的1500名年龄小于20岁的恶性肿瘤患者。数据收集基于一份经过验证的问卷调查,包括人口统计数据、临床数据和恶性肿瘤类型以及结果。采用SPSS软件22版对收集的数据进行定性和定量分析(P < 0.05)。生存率采用Kaplan-Meyer法计算。结果:本研究涉及1500名儿童,平均年龄6.1岁。最常见的恶性肿瘤是急性白血病(30.7%),其次是中枢神经系统肿瘤(27%)。在开始治疗时,复发、转移和继发恶性肿瘤的发生率分别为29%、19.5%和1%。52例患者行骨髓移植,其中死亡14例。3年、5年、10年总生存率分别为67.7%±0.01、60.3%±0.01、53.8%±0.01。结论:在伊朗建立以人群为基础的儿童癌症登记处是必要的,这将有助于提高上述患者的生存率。
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International Journal of Hematology-Oncology and Stem Cell Research
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