Hémopathies malignes

  • 文章类型: Review
    目的:血液恶性肿瘤的特殊性在于它们通常可以治愈,即使诊断时存在远处转移,不像实体恶性肿瘤.系统性治疗,包括化疗,靶向治疗,和免疫疗法,是护理的标准与优异的结果。在过去的20年中,这些疾病的管理取得了长足的进步,重新定义了放射治疗在许多临床情况下的作用。我们提出了对数据的文献综述,表明放射治疗仍然有治愈作用,打捞,和姑息治疗情况。
    方法:在以下数据库中进行了文档和文献检索:Medline和ClinicalTrial.gov,术语“放射治疗”,“血液恶性肿瘤”,“霍奇金淋巴瘤”,“非霍奇金淋巴瘤”,“CART细胞”,“多发性骨髓瘤”,“孤立性浆细胞瘤”,“调强放疗”,“颅外立体定向身体放射治疗”和“质子治疗参考”。
    结果:血液病理学恶性肿瘤包括多种疾病,在过去的20年中已经评估了放射治疗的适应症。目前,放射治疗适用于局部疾病(孤立性浆细胞瘤),作为佐剂(霍奇金淋巴瘤),在姑息环境中,或在复发患者(嵌合抗原受体[CAR]T细胞)的全身治疗后,复发负担低,因此,这可以被认为是“寡复发”。放射治疗,通过全身照射,有重要的适应症,由于其免疫调节和/或清髓作用。此外,最近的技术发展使得安全性有可能显著提高,有助于放射治疗被定位在几个适应症的治疗策略中。
    结论:鉴于系统治疗血液系统恶性肿瘤的有效性,寡转移阶段的重要性不大。局部放射治疗后的治愈意图,即使是高级阶段,是可能的,都患有晚期霍奇金淋巴瘤的残留病,侵袭性非霍奇金淋巴瘤,或者孤立性浆细胞瘤,甚至在霍奇金或非霍奇金淋巴瘤化疗后没有疾病的证据。新疗法的作用,例如CAR-T细胞,允许我们在全身治疗低体积复发的复发性疾病后考虑放射治疗,这可以被认为是少复发。
    OBJECTIVE: Haematologic malignancies are particular in that they can generally be cured, even when distant metastases are present at diagnosis, unlike solid malignancies. Systemic treatments, including chemotherapy, targeted therapies, and immunotherapy, are the standard of care with excellent results. The considerable progress made in the management of these diseases in the last 20years has redefined the role of radiation therapy as minor in many clinical situations. We propose a literature review of data, showing that radiation therapy still has a role in curative, salvage, and palliative therapy situations.
    METHODS: A document and literature search was carried out in the following databases: Medline and ClinicalTrial.gov, for the terms \"radiotherapy\", \"haematologic malignancies\", \"Hodgkin lymphoma\", \"non-Hodgkin lymphoma\", \"CAR T cells\", \"multiple myeloma\", \"solitary plasmocytoma\", \"intensity-modulated radiotherapy\", \"extracranial stereotactic body radiation therapy\" and \"proton therapy references\".
    RESULTS: Haemopathological malignancies include a wide range of diseases and radiation therapy indications have been assessed over the past 20years. Currently, radiation therapy is indicated for localized disease (solitary plasmocytoma), as an adjuvant (Hodgkin lymphoma), in palliative settings, or after systemic treatment in relapsed patients (chimeric antigen receptor [CAR] T-cells) with a low recurrence burden, which can therefore be considered \"oligorecurrence\". Radiation therapy, through total body irradiation, has important indications, thanks to its immunomodulatory and/or myeloablative effects. Moreover, recent technological developments have made possible significant improvement in safety, contributing to radiation therapy being positioned in the treatment strategy of several indications.
    CONCLUSIONS: Given the effectiveness of systemic treatments in hematologic malignancies, the oligometastasis stage is of little importance. A curative intent after local radiation therapy, even advanced stage, is possible, both with residual disease for advanced Hodgkin lymphoma, aggressive non-Hodgkin lymphoma, or solitary plasmocytoma, and even without evidence of disease after chemotherapy for Hodgkin or non-Hodgkin lymphoma. The role of new treatments, such as CAR T cells, allows us to consider radiation therapy after systemic treatment of relapsed diseases with low volume recurrence, which can be considered oligorecurrence.
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  • 文章类型: English Abstract
    新技术的出现使更频繁地识别骨髓增生异常综合征(MDS)和急性白血病(AL)的遗传易感性成为可能。目前最常见和最有特点的是CEBPA突变,RUNX1、GATA2、ETV6和DDX41,在存在这些具有高等位基因频率的突变之一的情况下,或在个人或家族史提示血液异常的情况下,如非免疫性血小板减少症,建议寻找遗传性血液系统恶性肿瘤(HHM)的可能性。的确,对这些HHM的早期识别可以更好地适应患者及其亲属的管理,因为异基因造血干细胞移植(HSCT)经常被提出用于这些病理。根据目前的数据,除了GATA2突变,突变的体质或体细胞性质似乎并不影响血液病的预后。因此,同种异体移植物的适应症将根据通常的标准确定。然而,在寻找家庭捐赠者时,重要的是要确保捐赠者没有遗传性疾病。为了保证在短时间内进行HSC同种异体移植的可能性,可能有必要启动平行程序以寻找无关的供体.鉴于在这种情况下HSC移植方式的信息有限,重要的是评估疾病的获益/风险以及决定条件处理类型(清髓性或降低强度)的程序.鉴于中期和长期继发性癌症风险的经验有限,建议降低强度调理可能是合适的,如在更好表征的综合征性血液系统疾病如范可尼贫血或端粒疾病的情况下。总之,更频繁地唤起HHM似乎很重要,特别是在有家族史的情况下,某些突变或持续性血液异常,为了讨论HSC同种异体移植的具体模式,特别是在寻找捐助者和评估程序的某些方式方面,比如调理。应该注意的是,HHM的发现,特别是如果同种异体HSC移植的迹象被保留,将提高伦理和心理的考虑,不仅为病人,还有他的家人.涉及分子生物学家的多学科方法,遗传学家,血液学家和心理学家是必不可少的。
    The advent of new technologies has made it possible to identify genetic predispositions to myelodysplastic syndromes (MDS) and acute leukemias (AL) more frequently. The most frequent and best characterized at present are mutations in CEBPA, RUNX1, GATA2, ETV6 and DDX41 and, either in the presence of one of these mutations with a high allelic frequency, or in the case of a personal or family history suggestive of blood abnormalities such as non-immune thrombocytopenia, it is recommended to look for the possibility of a hereditary hematological malignancy (HHM). Indeed, early recognition of these HHMs allows better adaptation of the management of patients and their relatives, as allogeneic hematopoietic stem cell transplantation (HSCT) is very often proposed for these pathologies. According to current data, with the exception of the GATA2 mutation, the constitutional or somatic nature of the mutations does not seem to influence the prognosis of hematological diseases. Therefore, the indication for an allograft will be determined according to the usual criteria. However, when searching for a family donor, it is important to ensure that there is no hereditary disease in the donor. In order to guarantee the possibility of performing the HSC allograft within a short period of time, it may be necessary to initiate a parallel procedure to find an unrelated donor. Given the limited information on the modalities of HSC transplantation in this setting, it is important to assess the benefit/risk of the disease and the procedure to decide on the type of conditioning (myeloablative or reduced intensity). In view of the limited experience with the risk of secondary cancers in the medium and long-term, it may be appropriate to recommend reduced intensity conditioning, as in the case of better characterized syndromic hematological diseases such as Fanconi anemia or telomere diseases. In summary, it seems important to evoke HHM more frequently, particularly in the presence of a family history, certain mutations or persistent blood abnormalities, in order to discuss the specific modalities of HSC allografting, particularly with regard to the search for a donor and the evaluation of certain modalities of the procedure, such as conditioning. It should be noted that the discovery of HHM, especially if the indication of an allogeneic HSC transplant is retained, will raise ethical and psychological considerations not only for the patient, but also for his family. A multidisciplinary approach involving molecular biologists, geneticists, hematologists and psychologists is essential.
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  • 文章类型: Journal Article
    BACKGROUND: End-of-life platelet transfusion practice in onco-hematology is subjective and depends on representations shared by patients, nurses and hematologists. This study aims to describe these representations of platelet transfusion in a context of a severe and advanced hematologic malignancy through the social representation of its protagonists.
    METHODS: A qualitative study, using the associative network method and including three groups of 15 participants (patients with an advanced hematologic malignancy, regularly transfused in platelet concentrates; nurses and hematologic oncologists) from four hematology centers was conducted between February and April 2019. Analysis was carried out using IraMuTeQ software.
    RESULTS: Patients expect platelet transfusion to have a direct beneficial impact on their health and highlight human relations. Nurses aim at the patient\'s well-being, in his or her individuality, and at respecting the transfusion protocol. Physicians seek to relieve symptoms by taking into account a multitude of decision-making factors. The textual clustering method, nuances those previous results and individualizes four different orientations, independent of groups: dependency, singularity, subjectivity and neutrality.
    CONCLUSIONS: The perception of the social representations related to platelet transfusion at the end-of-life should make it possible to adapt the discourse to the preferred orientation of the speaker and could be an asset in goals of care discussion with patients as well as with teams in charge of palliative care.
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  • 文章类型: Practice Guideline
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  • 文章类型: Journal Article
    Clonal hematopoiesis of undetermined significance or CHIP describes the identification, in individuals without hematologic disease, of one or more somatic mutations in hematopoietic cells. These mutations, detected by high-throughput genes sequencing (Next-Generation Sequencing or NGS), affect genes first identified in acute myeloid leukemia or myelodysplastic syndrome, such as DNMT3A, TET2 and ASXL1. CHIP is associated with an increased risk of malignant hemopathy, both myeloid and lymphoid, evaluated from 0.5 to 1% per year. CHIP is also associated with an increased risk of overall mortality and cardiovascular diseases. CHIP detection using NGS is currently limited to basic science field, but recent studies suggest that it may be of clinical interest.
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  • 文章类型: Journal Article
    UNASSIGNED: CURRENT USE IN THE UNITED STATES IN 2018: Treatment with T-cells engineered with chimeric antigen receptors (CAR T-cell therapy) has been a field of intense research in the United States since the 1980s. The recent approval in August 2017 of Kymriah (tisagenlecleucel) opened the door to broader access to CAR T-cell therapy outside of clinical trials. Here, we aim to give the reader a practical summary of the current practices in the US when considering a patient for CAR T-cell therapy. Cet article fait partie du numéro supplément Les cellules CAR-T : une révolution thérapeutique ? réalisé avec le soutien institutionnel des partenaires Gilead : Kite et Celgene.
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  • 文章类型: Journal Article
    BACKGROUND: The classification of hematological malignancies (HMs) has changed in recent decades. For the first time, the French network of cancer registries (Francim) provides estimates for incidence and trends of HM in France between 1980 and 2012 for major HM subtypes.
    METHODS: Incidence was directly estimated by modeling the incidence rates measured in the cancer registry area. For each HM subtype, a \"usable incidence period\" was defined a priori, corresponding to the years for which all the registries collected them in a homogeneous way. For both sexes and each HM subtype, age-period-cohort models were used to estimate national incidence trends.
    RESULTS: Overall in France, there were an estimated 35,000 new HMs in 2012 (19,400 in men and 15,600 in women). Lymphoid malignancies accounted for more than two-thirds of HM incident cases (n=25,136). The incidence sex ratio (M/F) varied from 1.1 for classical Hodgkin lymphoma to 4.0 for mantle-cell lymphoma. The median age at diagnosis ranged from 62 to 81 years according to the major HM subtypes. Overall in both sexes, the top five most frequent HMs in 2012 were plasma cell neoplasm (about 4900 estimated cases), chronic lymphocytic leukemia/small lymphocytic lymphoma (4500 cases), diffuse large B-cell lymphoma and myelodysplastic syndromes (4100 cases), and acute myeloid leukemia (2800 cases). The incidence rates increased for follicular lymphoma and plasma cell neoplasm during the study period in both sexes. Classical Hodgkin lymphoma was relatively stable in men between 1980 and 2012 and increased in both sexes during the most recent period. Chronic myeloproliferative neoplasms, other than chronic myelogenous leukemia, are the only subtype that showed a slightly downward trend in incidence between 2003 and 2012 in both sexes.
    CONCLUSIONS: The striking differences in the incidence patterns by histologic subtype strongly suggest a certain level of etiologic heterogeneity among hematological malignancies and support the pursuit of epidemiologic analysis by subtype for HMs in international studies. Age-standardized incidence rates are essential to analyze trends in risk, whereas the number of incident cases is necessary to make provisions for healthcare resources and to evaluate the overall burden of HM.
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  • 文章类型: English Abstract
    The platelet refractoriness is a complication of transfusion treatments potentially dramatic in onco-haematology. Chemo-treatment of haematological malignancies or packs of allogeneic bone marrow transplants require iterative platelet transfusion requirements. The discovery of a platelet refractoriness along with its support should be the most reactive as possible but also adapted to the cause. In the case of allo-immunization, it may be expected. The purpose of this presentation is to recall the different etiologies and perform a feedback on the support transfusion platelet of onco-haematology adult patients at Institut Paoli-Calmettes (IPC) in partnership with the EFSAM.
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  • 文章类型: Letter
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