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The Clinical Impact of Precisely Defining Mantle Cell Lymphoma: Contributions of Elaine Jaffe 精确定义套细胞淋巴瘤的临床影响:Elaine Jaffe的贡献
Hemato Pub Date : 2022-08-16 DOI: 10.3390/hemato3030035
M. Roschewski, D. Longo
{"title":"The Clinical Impact of Precisely Defining Mantle Cell Lymphoma: Contributions of Elaine Jaffe","authors":"M. Roschewski, D. Longo","doi":"10.3390/hemato3030035","DOIUrl":"https://doi.org/10.3390/hemato3030035","url":null,"abstract":"Mantle cell lymphoma (MCL) is an aggressive yet incurable B-cell lymphoma that was only first recognized as a distinct subtype in 1992, with early reports suggesting a poor median survival. Elaine Jaffe is a renowned hematopathologist and scientist from the National Cancer Institute who was instrumental in many of the early descriptions of MCL that distinguished it from other B-cell lymphomas. Further, she has led multiple international collaborations that have harmonized the lymphoma classification systems that are currently in use today. The early morphologic descriptions of MCL along with the contributions of immunologic and genetic techniques have confirmed MCL as a distinct entity with unique biology and clinical behavior. Importantly, these scientific discoveries laid the foundation for unprecedented therapeutic breakthroughs that have led to significant improvements in overall survival.","PeriodicalId":93705,"journal":{"name":"Hemato","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-08-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42259238","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: 0
The Era of Genomic Research for Lymphoma: Looking Back and Forward 淋巴瘤基因组研究时代的回顾与展望
Hemato Pub Date : 2022-08-15 DOI: 10.3390/hemato3030034
W. C. Chan, J. Iqbal
{"title":"The Era of Genomic Research for Lymphoma: Looking Back and Forward","authors":"W. C. Chan, J. Iqbal","doi":"10.3390/hemato3030034","DOIUrl":"https://doi.org/10.3390/hemato3030034","url":null,"abstract":"Technological and informatics advances as well as the availability of well-annotated and reliable genomic data have ushered in the era of genomics research. We describe in this brief review how the genomics approach has impacted lymphoma research in the understanding of the pathogenesis and biology of lymphoma, in lymphoma diagnosis and in targeted therapy. Some exciting directions that could be explored in the future are also discussed.","PeriodicalId":93705,"journal":{"name":"Hemato","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43083712","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: 0
Exploring Psychological Needs and Burden of Care in Parents of Children with Hemato-Oncological Diseases 血液肿瘤患儿家长的心理需求与照顾负担探讨
Hemato Pub Date : 2022-08-01 DOI: 10.3390/hemato3030033
Loredana Benedetto, I. Marino, F. Ronco, G. Iaria, Luisa Foletti, M. Ingrassia
{"title":"Exploring Psychological Needs and Burden of Care in Parents of Children with Hemato-Oncological Diseases","authors":"Loredana Benedetto, I. Marino, F. Ronco, G. Iaria, Luisa Foletti, M. Ingrassia","doi":"10.3390/hemato3030033","DOIUrl":"https://doi.org/10.3390/hemato3030033","url":null,"abstract":"Caring for a child with an acute/life threatening disease exposes parents to multiple stressors and challenges, resulting in a physical and psychological burden. Parents experience many health-related issues and worries that often remain underestimated. The aims of the study were: (a) to explore the associations between needs/disease-related issues and burden in parents of children with leukemia or Hodgkin’s disease; (b) to estimate predictors of parents’ burden using a stepwise linear regression analysis. Children (N = 33) followed an active therapy protocol (48.5%), or they were off therapy (51.5%). Forty-four parents completed surveys on caregiver burden levels and needs to cope with the child’s illness. Parental factors impacting burden (personal resources, loss of control, depression) and child’s quality of life (QoL) were also assessed. Among the needs, information about the illness/resources were the most urgently expressed by parents, followed by reassurance against fears for the child’s development and future well-being. Parents reported severe (27.3%) and moderate (22.7%) burden, with a higher percentage of caregivers with severe burden in the off-therapy phase (18.2%) than in the active-therapy phase (9.1%). The child’s decreased physical QoL and parent’s loss of control predicted higher levels of burden. The implications for supportive interventions aimed at responding to parental needs and preventing caregiver burden are discussed.","PeriodicalId":93705,"journal":{"name":"Hemato","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47572614","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: 2
Evolution in the Definition of Follicular Lymphoma and Diffuse Large B-Cell Lymphoma: A Model for the Future of Personalized Medicine 滤泡性淋巴瘤和弥漫性大B细胞淋巴瘤定义的演变:个性化医学的未来模型
Hemato Pub Date : 2022-07-21 DOI: 10.3390/hemato3030032
E. Jaffe, A. Carbone
{"title":"Evolution in the Definition of Follicular Lymphoma and Diffuse Large B-Cell Lymphoma: A Model for the Future of Personalized Medicine","authors":"E. Jaffe, A. Carbone","doi":"10.3390/hemato3030032","DOIUrl":"https://doi.org/10.3390/hemato3030032","url":null,"abstract":"The definitions of follicular lymphoma (FL) and diffuse large B-cell lymphoma (DLBCL) are evolving in the era of personalized medicine. Early stages of the evolution of FL have been recognized. Two histological manifestations of early lesions are in situ follicular neoplasia and duodenal type FL. Additionally, FL frequently undergoes histological transformation, the most common form being DLBCL. High-grade B-cell lymphoma with double hit, with translocations involving BCL2 and MYC are important clinically. Rarer forms of transformation include classic Hodgkin lymphoma (CHL) and histiocytic sarcoma. In addition to conventional FL associated with the BCL2 translocation, alternative forms of BCL2-negative FL have been observed. These are heterogenous clinically and genetically. A distinctive group of B-cell lymphomas of follicle cell derivation arise in young patients and include pediatric type FL, testicular FL and a large B-cell lymphoma with IRF4 rearrangement. Historically DLBCL was separated into only two histological variants, centroblastic and immunoblastic. In 2017 the WHO classification recommended (1) the segregation of activated B cell and germinal center B cell derived DLBCL, (2) the identification of high-grade B-cell lymphoma with double hit, and (3) the recognition of an aggressive lymphoma that may resemble Burkitt lymphoma, currently designated in the International Consensus Classification as Large B-cell lymphoma with 11q aberration. Today we appreciate greater genomic complexity among aggressive B-cell lymphomas. Recent studies with NGS and mutational profiling have identified clinically significant genetic subgroups. It is hoped that these data ultimately will lead to targeted therapy based on the genetic profile.","PeriodicalId":93705,"journal":{"name":"Hemato","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48591373","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: 2
Minimal Residual Disease in Multiple Myeloma—Current Approaches and Future Clinical Implications 多发性骨髓瘤的微小残留疾病:目前的方法和未来的临床意义
Hemato Pub Date : 2022-07-19 DOI: 10.3390/hemato3030031
T. Akhlaghi, Ross Firestone, M. Hultcrantz
{"title":"Minimal Residual Disease in Multiple Myeloma—Current Approaches and Future Clinical Implications","authors":"T. Akhlaghi, Ross Firestone, M. Hultcrantz","doi":"10.3390/hemato3030031","DOIUrl":"https://doi.org/10.3390/hemato3030031","url":null,"abstract":"The prognosis and clinical outcomes for patients with multiple myeloma have improved significantly over the past two decades. A substantial number of patients now achieve complete remission after induction therapy, and more sensitive methods are needed to assess response. Minimal or measurable residual disease (MRD) has been incorporated in many clinical trials as well as in clinical practice. The importance of MRD assessment and correlation between MRD negativity and prolonged progression-free and overall survival has been confirmed in numerous clinical trials and several meta-analyses. Recent studies have even suggested that MRD negativity can partly overcome the impact of the negative prognostic factors such as high-risk cytogenetics or adverse revised international scoring system (R-ISS) stage. MRD can be measured in the bone marrow via imaging and via emerging blood-based techniques. The most common methods are multicolor flow cytometry and next-generation sequencing of bone marrow samples. Using these methods in optimal settings, MRD negativity with a sensitivity level of 10−6 can be detected. In this review, we discuss the benefits and limitations of these techniques as well as the clinical implications.","PeriodicalId":93705,"journal":{"name":"Hemato","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42024066","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
Burkitt Lymphoma Incidence in Five Continents 五大洲的伯基特淋巴瘤发病率
Hemato Pub Date : 2022-07-13 DOI: 10.3390/hemato3030030
S. Mbulaiteye, S. Devesa
{"title":"Burkitt Lymphoma Incidence in Five Continents","authors":"S. Mbulaiteye, S. Devesa","doi":"10.3390/hemato3030030","DOIUrl":"https://doi.org/10.3390/hemato3030030","url":null,"abstract":"Burkitt lymphoma (BL) is a rare non-Hodgkin lymphoma first described in 1958 by Denis Burkitt in African children. BL occurs as three types, endemic, which occurs in Africa and is causally attributed to Epstein-Barr virus and P. falciparum infections; sporadic, which occurs in temperate areas, but the cause is obscure; and immunodeficiency-type, which is associated with immunosuppression. All BL cases carry IG∷MYC chromosomal translocations, which are necessary but insufficient to cause BL. We report a comprehensive study of the geographic, sex, and age-specific patterns of BL among 15,122 cases from Cancer Incidence in Five Continents Volume XI for 2008–2012 and the African Cancer Registry Network for 2018. Age-standardized BL rates were high (>4 cases per million people) in Uganda in Africa, and Switzerland and Estonia in Europe. Rates were intermediate (2–3.9) in the remaining countries in Europe, North America, and Oceania, and low (<2) in Asia. Rates in India were 1/20th those in Uganda. BL rates varied within and between regions, without showing a threshold to define BL as endemic or sporadic. BL rates were twice as high among males as females and showed a bimodal age pattern with pediatric and elderly peaks in all regions. Multi-regional transdisciplinary research is needed to elucidate the epidemiological patterns of BL.","PeriodicalId":93705,"journal":{"name":"Hemato","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-07-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43326040","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: 5
Role of PPAR Receptor and Ligands in the Pathogenesis and Therapy of Hematologic Malignancies PPAR受体和配体在血液恶性肿瘤发病和治疗中的作用
Hemato Pub Date : 2022-06-30 DOI: 10.3390/hemato3030029
J Wu, M. Zhang, Allison Faircloth
{"title":"Role of PPAR Receptor and Ligands in the Pathogenesis and Therapy of Hematologic Malignancies","authors":"J Wu, M. Zhang, Allison Faircloth","doi":"10.3390/hemato3030029","DOIUrl":"https://doi.org/10.3390/hemato3030029","url":null,"abstract":"The Peroxisome proliferator-activated receptors (PPARs) play vital roles in regulating cellular differentiation, proliferation, and caspase-mediated cell death pathways. They are regarded as promising targets for anti-tumor drug development, particularly for multiple myeloma (MM) and different hematological malignancies. Several early section clinical trials are conducted to measure the clinical practicableness of PPAR agonists, notably PPARα and PPARγ agonists, against various cancers. A spread of studies has investigated PPARs expression in metabolic regulation. Furthermore, it has been suggested that careful designing of partial agonists for PPARs may show improvement with side effects and increase the therapeutic value. This review summarizes the organic chemistry and metabolic actions of PPARs, and the therapeutic potential of their agonists underneath clinical development. It investigates therapeutic agents for hematologic malignancies.","PeriodicalId":93705,"journal":{"name":"Hemato","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49171250","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: 0
Measurable Residual Disease Assessment in Multiple Myeloma: How Deep Is Enough? 多发性骨髓瘤可测量残余病变评估:多深才够?
Hemato Pub Date : 2022-06-23 DOI: 10.3390/hemato3030027
Joana Caetano, F. Barahona, P. Lúcio, C. João
{"title":"Measurable Residual Disease Assessment in Multiple Myeloma: How Deep Is Enough?","authors":"Joana Caetano, F. Barahona, P. Lúcio, C. João","doi":"10.3390/hemato3030027","DOIUrl":"https://doi.org/10.3390/hemato3030027","url":null,"abstract":"The introduction of new and more effective therapeutic options for Multiple Myeloma (MM) has significantly deepened and prolonged patients’ remission. As currently used treatment protocols induce high rates of complete responses, Measurable Residual Disease (MRD) assessment has become essential to enhance the evaluation of treatment efficacy. Detection of MRD has improved with the development of highly sensitive and standardized techniques such as Next Generation Flow or Next Generation Sequencing, complemented by functional imaging techniques. These advances offer a valuable opportunity to further optimize criteria of response to treatment. Currently, extensive data demonstrate that MRD status is a valuable prognostic factor of survival. Since MRD represents a real measurement of disease burden, its incorporation in clinical trials to guide treatment decisions will certainly translate into clinical benefits. Sustained MRD negativity can be used to consider optimal candidates for treatment discontinuation, whereas MRD positive high-risk patients may have access to novel immunotherapeutic strategies such as bispecific drugs or CAR T cell therapy. In this review, we describe the available techniques to detect MRD, address the current data regarding MRD as a surrogate endpoint within clinical trials, examine how MRD can be introduced into the clinical management of MM patients, and discuss the future of MRD monitoring.","PeriodicalId":93705,"journal":{"name":"Hemato","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-06-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46204238","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
Functional Impairments of Amyloidosis Patients: Physical Therapy Assessment 淀粉样变性患者的功能损害:物理治疗评估
Hemato Pub Date : 2022-06-23 DOI: 10.3390/hemato3030028
E. Redder, Qiuhong Zhao, N. Bumma, R. Kahwash, A. Vallakati, Courtney M. Campbell, Samir M. Parikh, S. Almaani, M. Freimer, Y. Efebera, Nidhi Sharma
{"title":"Functional Impairments of Amyloidosis Patients: Physical Therapy Assessment","authors":"E. Redder, Qiuhong Zhao, N. Bumma, R. Kahwash, A. Vallakati, Courtney M. Campbell, Samir M. Parikh, S. Almaani, M. Freimer, Y. Efebera, Nidhi Sharma","doi":"10.3390/hemato3030028","DOIUrl":"https://doi.org/10.3390/hemato3030028","url":null,"abstract":"Amyloidosis is a rare, systemic disease that can result in significant functional impairment. Specific guidelines for the rehabilitation assessment of amyloidosis patients have yet to be established. The purpose of this study was to identify functional deficits and assess differences based on disease type, organ involvement, age, and gender of patients with amyloidosis. Materials and Methods: The multidisciplinary Comprehensive Amyloidosis Clinic (CAC) at Ohio State University (OSU) has developed structured assessment guidelines for amyloidosis patients. A retrospective, single-institution review of patients assessed in CAC between December 2017 and April 2020 was performed. Outcome measure data from the Timed Up and Go (TUG), 30 s sit-to-stand, and physical function portion of the SF 36 were gathered by chart review. Comparisons were made between CAC patient scores and normative data. Kruskal–Wallis tests were used to compare scores across the disease types (light chain, transthyretin wild-type, and hereditary variant transthyretin) and the Mann–Whitney U test was used for pairwise comparisons within disease types and cardiac involvement. Linear regression models were used to assess associations between patient characteristics (including age, gender, disease type, and cardiac involvement) and performance scores. Results: Data from sixty-four patients was evaluated. On the 30-s sit-to-stand test, patients with light chain amyloidosis performed 3.32 fewer repetitions than patients with transthyretin wild-type, p = 0.03. Patients with cardiac involvement had 2.55 fewer repetitions than patients without cardiac involvement, p = 0.03. Older patients were found to have slower TUG performance, and a 10-year increase in age was associated with an 11% increase in TUG scores. Conclusions: Findings indicate patients with light chain amyloidosis and patients with cardiac involvement, when compared to other amyloidosis patients, present with more physical impairments.","PeriodicalId":93705,"journal":{"name":"Hemato","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-06-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45447235","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: 0
miR-494 in Extracellular Vesicles as a Potent Biomarker of Chronic Myeloid Leukemia Treatment with Tyrosine Kinase Inhibitors 细胞外小泡中的miR-494作为酪氨酸激酶抑制剂治疗慢性粒细胞白血病的有效生物标志物
Hemato Pub Date : 2022-06-13 DOI: 10.3390/hemato3020026
Tatsuki Shibuta, Honoka Shimizu, Yukichi Takada, Asuka Fuku, Satoshi Tomiyasu, T. Umemura
{"title":"miR-494 in Extracellular Vesicles as a Potent Biomarker of Chronic Myeloid Leukemia Treatment with Tyrosine Kinase Inhibitors","authors":"Tatsuki Shibuta, Honoka Shimizu, Yukichi Takada, Asuka Fuku, Satoshi Tomiyasu, T. Umemura","doi":"10.3390/hemato3020026","DOIUrl":"https://doi.org/10.3390/hemato3020026","url":null,"abstract":"Extracellular vesicles (EVs) are nano-sized particles released from cells and transferring molecules (proteins, lipids and nucleic acids such as mRNA, tRNA and miRNA) to recipient cells. Surface antigens and components are important for the functions as cell-to-cell communication of EVs. Thus, EVs are useful biomarkers for various diseases including leukemias and other types of malignancies. We evaluated whether miRNAs in EVs released from chronic myelogenous leukemia (CML) cells could be used for diagnosis. Microarray analysis of miRNAs in EVs obtained from the culture supernatants of two CML cell lines showed that miR-494 and miR-373-5p were significantly decreased by tyrosine kinase inhibitor for BCR-ABL1. Validation analysis with Taqman-based qRT-PCR of whole serum obtained patients with CML in the chronic phase (n = 5) did not show a significant difference in miR-494 levels compared to the CML accelerated phase and blast crisis patients (n = 5). However, the levels of miR-494 were 2.9-fold higher in the accelerated phase or blast crisis than in the chronic phase (p < 0.05). These results indicate that it is important to measure miR-494 using only EVs rather than whole serum. Our data suggest that EV-miR-494 is a useful biomarker of CML progression and evaluation of response to tyrosine kinase inhibitors.","PeriodicalId":93705,"journal":{"name":"Hemato","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49523072","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: 0
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