EMC - HématologiePub Date : 2004-11-01Epub Date: 2004-09-25DOI: 10.1016/j.emch.2004.08.002
J. Dupuis , C. Gisselbrecht
{"title":"Lymphomes non hodgkiniens T et NK périphériques","authors":"J. Dupuis , C. Gisselbrecht","doi":"10.1016/j.emch.2004.08.002","DOIUrl":"10.1016/j.emch.2004.08.002","url":null,"abstract":"<div><p>Peripheral T and NK-cell lymphomas constitute an heterogeneous group of lymphoid neoplasms. They represent approximately 15% of lymphomas in Western countries. Their prognosis is unfavourable as compared to B cell lymphomas. The WHO classification identifies no less than sixteen different entities. Anaplastic lymphoma, angio-immunoblastic lymphoma and T-cell lymphoma not otherwise specified represent the three main groups. Each of these possesses its own epidemiological, physio-pathological, prognostic and therapeutic characteristics. There is no established specific chemotherapy for most of these entities, but novel therapeutic approaches are in progress.</p></div>","PeriodicalId":100425,"journal":{"name":"EMC - Hématologie","volume":"1 4","pages":"Pages 135-149"},"PeriodicalIF":0.0,"publicationDate":"2004-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.emch.2004.08.002","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87244819","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}
EMC - HématologiePub Date : 2004-11-01Epub Date: 2004-09-21DOI: 10.1016/j.emch.2004.08.003
P. Rachieru (Praticien attaché) , A. Schmidt (Assistante hospitalo-universitaire) , I. Pellier (Praticien hospitalier) , A. Godon (Assistant hospitalo-universitaire) , V. Daniel (Pharmacien hospitalier) , X. Rialland (Praticien hospitalier) , M. Hunault-Berger (Professeur des Universités, Praticien hospitalier) , N. Ifrah (Professeur des Universités, praticien hospitalier, chef de service)
{"title":"Utilisation thérapeutique des immunoglobulines polyvalentes en hématologie","authors":"P. Rachieru (Praticien attaché) , A. Schmidt (Assistante hospitalo-universitaire) , I. Pellier (Praticien hospitalier) , A. Godon (Assistant hospitalo-universitaire) , V. Daniel (Pharmacien hospitalier) , X. Rialland (Praticien hospitalier) , M. Hunault-Berger (Professeur des Universités, Praticien hospitalier) , N. Ifrah (Professeur des Universités, praticien hospitalier, chef de service)","doi":"10.1016/j.emch.2004.08.003","DOIUrl":"10.1016/j.emch.2004.08.003","url":null,"abstract":"<div><p>The efficacy and the safety of intravenous immunoglobulins (IVIG) have been attentively explored since 1970. Their mechanisms of action involve the Fc-receptor blockade, the neutralization of microbial toxins, and the modulation of cytokine production. Their indications depend both on their replacement and immuno-modulation abilities. They include primary antibody deficiencies, secondary hypogammaglobulinemic states with serious repetitive infections, and autoimmune disorders such as the immune thrombocytopenic purpura, whereas IVIG are no more systematically used in sibling hematopoietic stem cell transplantation. Despite major precautions in the manufacturing process, there is still a possible transmission of hitherto unrecognized or recognized infective agents for blood-derived preparations. This emphasizes the need of excellent indications for these expensive products.</p></div>","PeriodicalId":100425,"journal":{"name":"EMC - Hématologie","volume":"1 4","pages":"Pages 150-163"},"PeriodicalIF":0.0,"publicationDate":"2004-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.emch.2004.08.003","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76434533","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}
EMC - HématologiePub Date : 2004-08-01Epub Date: 2004-09-15DOI: 10.1016/j.emch.2004.05.001
D. Decaudin (MD, PhD)
{"title":"Lymphome du manteau : un modèle biologique et clinique","authors":"D. Decaudin (MD, PhD)","doi":"10.1016/j.emch.2004.05.001","DOIUrl":"10.1016/j.emch.2004.05.001","url":null,"abstract":"<div><p>Recent classifications of non-Hodgkin’s lymphomas (NHL) have strictly individualized mantle cell lymphoma (MCL) on the basis of a combination of morphologic, immunophenotypic, and cytogenetic criteria. This clinicopathological entity now appears to be a biological and therapeutic model for the understanding and treatment of hematological malignancies. The lymphomogenesis of MCL could be explained by a series of genetic abnormalities which occur at different steps of the disease: (1) mutation and/or loss of the <em>ATM</em> gene in centrocytic cells of the follicle mantle of lymph nodes, leading to the loss of ATM function, particularly involved during the <em>(D) J</em> recombination process; (2) a (11 ; 14) (q13 ; q32) translocation which induces a constitutive Bcl-1/PRAD1/CCND1 expression, responsible for cell cycle activation of centrocytic cells characteristic of typical MCL; and (3) secondary additional chromosomal aberrations, such as a <em>p53</em> mutation, observed in blastic transformation of MCL. Despite the evaluation of a number of treatment modalities, the optimal management of MCL has not been defined yet; (1) conventional and intensified chemotherapy and monoclonal anti-CD20 antibody therapy appear to be effective for the improvement of response rates and event-free or overall survival; (2) combinations of different treatment modalities must be tested to modify the natural dismal outcome of the disease; (3) innovative approaches should be developed. From this point of view, all these considerations offer a fine opportunity for extensive medical reflection.</p></div>","PeriodicalId":100425,"journal":{"name":"EMC - Hématologie","volume":"1 3","pages":"Pages 69-82"},"PeriodicalIF":0.0,"publicationDate":"2004-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.emch.2004.05.001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88674529","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}
EMC - HématologiePub Date : 2004-08-01Epub Date: 2004-07-23DOI: 10.1016/j.emch.2004.06.002
J.-P. Marie , C. Marzac , O. Legrand
{"title":"Mécanismes de résistance aux agents cytostatiques","authors":"J.-P. Marie , C. Marzac , O. Legrand","doi":"10.1016/j.emch.2004.06.002","DOIUrl":"10.1016/j.emch.2004.06.002","url":null,"abstract":"<div><p>Most of the time, the mechanisms of drug resistance in tumoral cells are multifactorial. The most studied mechanism is the MDR (multi-drug resistance) phenotype, because it is related to major cytostatic drugs like anthracyclins, vinca-alkaloïds and epipodophyllotoxins. MDR is due to the presence of transporter proteins (ABC proteins) that are able to expel drugs of natural origin (xenobiotics). The presence of P-gp, the most studied protein of this family, is of value for predicting drug resistance in acute myeloid leukemia. This explains the use of “modulators” of P-gp like quinine, cyclosporine or PSC833 in this disease. A benefit for the patient is observed only in case of functional P-gp present on the leukemic cells. The role of the other ABC pumps (MRPs, BCRP) is not clearly demonstrated in hematological malignancies. Another important mechanism of resistance is the inhibition of drug-induced apoptosis, due to modification of p53 or proteins of the bcl-2 family. Enzyme modifications are also important in resistance to nucleotide analogs like cytosine arabinoside, largely used in acute leukemia. The use of doses ten-fold higher than the standard dosing allows overcoming resistance to this drug.</p></div>","PeriodicalId":100425,"journal":{"name":"EMC - Hématologie","volume":"1 3","pages":"Pages 59-68"},"PeriodicalIF":0.0,"publicationDate":"2004-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.emch.2004.06.002","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82629702","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}
EMC - HématologiePub Date : 2004-06-01Epub Date: 2004-06-02DOI: 10.1016/j.emch.2003.11.001
F Daffos, B Pedron-Grossetete, G Sterkens, V Mirlesse
{"title":"Données d'hématologie, d'hémostase et d'immunologie chez le fœtus normal","authors":"F Daffos, B Pedron-Grossetete, G Sterkens, V Mirlesse","doi":"10.1016/j.emch.2003.11.001","DOIUrl":"10.1016/j.emch.2003.11.001","url":null,"abstract":"<div><p>The development of techniques of fetal blood sampling has allowed significant progress in our knowledge of the physiologic values of hematologic parameters in the foetus. The first step is to assess the purity of the sample and absence of maternal contamination. The knowing of the normal values is a prerequisite for accurate diagnosis and adequate management.</p></div>","PeriodicalId":100425,"journal":{"name":"EMC - Hématologie","volume":"1 2","pages":"Pages 21-34"},"PeriodicalIF":0.0,"publicationDate":"2004-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.emch.2003.11.001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84070799","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}
EMC - HématologiePub Date : 2004-06-01Epub Date: 2004-06-02DOI: 10.1016/j.emch.2003.12.001
F. Daffos, F. Jacquemard, Y. Rouquet
{"title":"Techniques et indications hématologiques du prélèvement de sang fœtal et de la transfusion in utero","authors":"F. Daffos, F. Jacquemard, Y. Rouquet","doi":"10.1016/j.emch.2003.12.001","DOIUrl":"10.1016/j.emch.2003.12.001","url":null,"abstract":"<div><p>Fetal blood sampling has become available for routine use during the late twenty years of the past century. This invasive technique is highly useful and it is safe in experienced hands. In the field of fetal hematology, it allows or increases the accuracy of the diagnosis of various conditions. Therapeutic possibilities are open, mainly fetal transfusion. The technical aspects and current hematological indications of fetal blood access are reviewed.</p></div>","PeriodicalId":100425,"journal":{"name":"EMC - Hématologie","volume":"1 2","pages":"Pages 35-45"},"PeriodicalIF":0.0,"publicationDate":"2004-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.emch.2003.12.001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77881075","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}
EMC - HématologiePub Date : 2004-06-01Epub Date: 2004-06-02DOI: 10.1016/j.emch.2004.02.001
A Jaccard , J.-P Fermand
{"title":"Amyloses","authors":"A Jaccard , J.-P Fermand","doi":"10.1016/j.emch.2004.02.001","DOIUrl":"https://doi.org/10.1016/j.emch.2004.02.001","url":null,"abstract":"<div><p>The pathology in all forms of amyloidosis involves the extra cellular deposition of protein as characteristic fibrillar aggregates which interfere with tissue structure and function. Amyloid fibrils are derived from different unrelated proteins in the different forms of the disease. Amyloidosis may be hereditary or acquired, and the deposit distribution may be focal, localized or systemic. The diagnosis of amyloidosis requires histological confirmation usually with Congo red staining. Prognosis depends upon the degrees of visceral amyloid deposition, particularly in the heart. A diagnosis of amyloidosis should be followed by characterization of the fibril protein type in order to determine the appropriate management. The aim of treatment is to reduce the supply of the respective amyloid fibril precursor protein, which can result in major regression of deposits.</p></div>","PeriodicalId":100425,"journal":{"name":"EMC - Hématologie","volume":"1 2","pages":"Pages 46-58"},"PeriodicalIF":0.0,"publicationDate":"2004-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.emch.2004.02.001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"137222948","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}
EMC - HématologiePub Date : 2004-03-01Epub Date: 2004-05-27DOI: 10.1016/j.emch.2003.11.002
V Mirlesse , D Mitanchez
{"title":"Syndrome anémique fœtal","authors":"V Mirlesse , D Mitanchez","doi":"10.1016/j.emch.2003.11.002","DOIUrl":"10.1016/j.emch.2003.11.002","url":null,"abstract":"<div><p>Anaemia in the fetus is suspected either in conditions at risk or after the discovery of indirect signs, mainly on ultrasound examination. Fetal blood sampling ascertains the diagnosis and allows a precise evaluation of the degree of anaemia. The management of deep anaemia may include prenatal transfusion. Main etiologies and the possibilites of antenatal diagnosis are reviewed.</p></div>","PeriodicalId":100425,"journal":{"name":"EMC - Hématologie","volume":"1 1","pages":"Pages 2-8"},"PeriodicalIF":0.0,"publicationDate":"2004-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.emch.2003.11.002","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74701367","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}
EMC - HématologiePub Date : 2004-03-01Epub Date: 2004-05-27DOI: 10.1016/j.emch.2003.10.001
A Fischer
{"title":"Syndromes déficitaires immunologiques fœtaux","authors":"A Fischer","doi":"10.1016/j.emch.2003.10.001","DOIUrl":"10.1016/j.emch.2003.10.001","url":null,"abstract":"<div><p>Severe combined immunodeficiencies are the most common immunologic deficiencies that may involve prenatal or emergency perinatal management. Antenatal diagnosis is required for early management. Gene therapy raised great hopes but can lead to serious adverse events.</p></div>","PeriodicalId":100425,"journal":{"name":"EMC - Hématologie","volume":"1 1","pages":"Pages 18-20"},"PeriodicalIF":0.0,"publicationDate":"2004-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.emch.2003.10.001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83874403","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}
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