EMC - Hématologie最新文献_第9页

EMC - Hématologie Pub Date : 2005-06-01 DOI: 10.1016/j.emch.2005.01.001

C. Boyer-Neumann

{"title":"Hémostase et grossesse","authors":"C. Boyer-Neumann","doi":"10.1016/j.emch.2005.01.001","DOIUrl":"https://doi.org/10.1016/j.emch.2005.01.001","url":null,"abstract":"<div><p>Normal pregnancy is an acquired hypercoagulable and inflammatory state. Hypercoagulability in normal pregnancy is due to increased levels of procoagulant coagulation factors, as well as hypofibrinolysis and decreased levels of natural anticoagulants. In addition, regulation of the maternal immune system contributes to a successful pregnancy outcome. Specific pregnancy diseases are defined by their occurrence in relation with the gravid state and their recovery when gestation ends, after delivery, most of the time in emergency conditions in case of: disseminated intravascular coagulation, thrombotic microangiopathies, such as preeclampsia, HELLP syndrome, or the rare thrombotic thrombocytopenic purpura. On the contrary, diseases that enhance bleeding risk are present before pregnancy when hereditary, or detected at the time of pregnancy when acquired: hereditary diseases include von Willebrand disease, thrombopathies, or conducting state of haemophilia, and acquired states are mainly related to autoimmune diseases, such as immune thrombocytopenic purpura. Various corrections may be performed for pregnancy-induced abnormalities and their management and follow-up are achieved accordingly. Diseases that increase the thrombotic risk involve hereditary thrombophilia (deficiencies of natural anticoagulants, factors V or II mutations), and acquired thrombophilia (antiphospholipids antibodies). Normal pregnancy and post-partum also constitute an acquired thrombotic factor and women with thrombophilic defects have been shown to be at increased risk. There is a consensus that such women with a personal history of thrombosis should receive anticoagulant prophylaxis. A good evaluation of the thrombotic risk is necessary in order to determine the most appropriate anticoagulant treatment. Warfarin crosses the placenta and has a known teratogenic effect (warfarin embryopathy). Hence, prophylaxis of gestational thrombosis mainly relies upon the use of heparin, during pregnancy and post-partum.</p></div>","PeriodicalId":100425,"journal":{"name":"EMC - Hématologie","volume":"2 2","pages":"Pages 132-143"},"PeriodicalIF":0.0,"publicationDate":"2005-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.emch.2005.01.001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91712543","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: 7

Groupes sanguins érythrocytaires 红细胞血型

EMC - Hématologie Pub Date : 2005-06-01 DOI: 10.1016/j.emch.2005.04.001

J. Chiaroni (Docteur en Médecine, docteur d'Université) , V. Ferrera (Docteur en Pharmacie, biologiste, ancien interne des Hôpitaux, docteur d'Université) , I. Dettori (Docteur en Médecine, biologiste, ancien interne des Hôpitaux) , F. Roubinet (Docteur en Médecine, docteur d'Université)

{"title":"Groupes sanguins érythrocytaires","authors":"J. Chiaroni (Docteur en Médecine, docteur d'Université) , V. Ferrera (Docteur en Pharmacie, biologiste, ancien interne des Hôpitaux, docteur d'Université) , I. Dettori (Docteur en Médecine, biologiste, ancien interne des Hôpitaux) , F. Roubinet (Docteur en Médecine, docteur d'Université)","doi":"10.1016/j.emch.2005.04.001","DOIUrl":"https://doi.org/10.1016/j.emch.2005.04.001","url":null,"abstract":"<div><p>Red-cell blood groups can be defined as genetically transmitted allotypic variations that may be detected by specific antibodies on red cell surface. Most antigens can be classified in systems according to defined genetic criteria. Antigens that have not been sufficiently characterized are included in groups called collections or series. Although our understanding about the physiological functions of the molecules defining blood groups has improved, it remains much to be learned concerning the exact role of these factors on the erythrocyte and biological implications of polymorphism. Due to the immunogenic nature of their polymorphism, these systems produce conflicts in case of transfusion, pregnancy, and, sometimes, transplantation. These antigens can also be the targets of auto-antibodies causing autoimmune hemolytic anemia. Guidelines for transfusion compatibility and immuno-hematological follow-up during pregnancy are also based on the analysis of these antigens and antibodies. The purpose of this article is to describe the antigens, coding genes, and recognized antibodies involved in blood grouping. Each case will include an assessment of the implications for human disease and transfusion medicine. Whenever possible, functional aspects will be analyzed.</p></div>","PeriodicalId":100425,"journal":{"name":"EMC - Hématologie","volume":"2 2","pages":"Pages 53-112"},"PeriodicalIF":0.0,"publicationDate":"2005-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.emch.2005.04.001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91712544","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: 16

Aplasies médullaires acquises 获得性髓系发育不全

EMC - Hématologie Pub Date : 2005-06-01 DOI: 10.1016/j.emch.2005.01.002

G. Socié (Professeur, praticien universitaire-praticien hospitalier) , C. Ferry (Attaché) , M. Robin (Chef de clinique-assistante) , J.-Y. Mary (Directeur de recherche Inserm)

引用次数: 3

Inhibiteurs du fonctionnement plaquettaire 血小板功能抑制剂

EMC - Hématologie Pub Date : 2005-03-01 DOI: 10.1016/j.emch.2004.12.001

T. Lecompte (Docteur en médecine, praticien universitaire-praticien hospitalier en hématologie), M. Toussaint-Hacquard (Docteur en pharmacie, docteur d’université, assistant hospitalier universitaire en hématologie)

引用次数: 0

Morphologie des cellules sanguines normales 正常血细胞的形态

EMC - Hématologie Pub Date : 2005-03-01 DOI: 10.1016/j.emch.2004.10.001

F. Valensi

引用次数: 4

Purpura thrombotique thrombocytopénique et autres syndromes de microangiopathie thrombotique 血栓性紫癜血小板减少和其他血栓性微血管病综合征

EMC - Hématologie Pub Date : 2005-03-01 DOI: 10.1016/j.emch.2004.12.002

P. Coppo , J.-P. Vernant , A. Veyradier , V. Frémeaux-Bacchi , J.-P. Mira , B. Guidet , E. Azoulay , E. Rondeau , A. Bussel , pour le Réseau d’étude des microangiopathies thrombotiques de l’adulte

{"title":"Purpura thrombotique thrombocytopénique et autres syndromes de microangiopathie thrombotique","authors":"P. Coppo , J.-P. Vernant , A. Veyradier , V. Frémeaux-Bacchi , J.-P. Mira , B. Guidet , E. Azoulay , E. Rondeau , A. Bussel , pour le Réseau d’étude des microangiopathies thrombotiques de l’adulte","doi":"10.1016/j.emch.2004.12.002","DOIUrl":"10.1016/j.emch.2004.12.002","url":null,"abstract":"<div><p>Thrombotic microangiopathies (TMA) refer to various diseases characterized by microangiopathic hemolytic anemia, peripheral thrombocytopenia, and organ failure of variable severity. Microvascular thrombosis is the typical histopathological lesion. Thrombotic thrombocytopenic purpura (TTP) (or Moschowitz syndrome) and hemolytic uremic syndrome (HUS) represent the two most classical forms of TMA. TTP is typically characterized by a multiorgan involvement, whereas in HUS severe renal dysfunction is the major finding. However, a TMA may also be observed within a context of neoplasia, chemotherapy, hematopoietic stem cell transplantation, human immunodeficiency virus (HIV) infection, malignant hypertension, or HELLP syndrome. TMA are associated with a severe prognosis. Therefore, rapid diagnosis and appropriate treatment are required. Management of TMA consists in plasma exchanges, allowing the infusion of large volumes of plasma. In some patients, immunosuppressive drugs are associated. Recently, major findings led to a better understanding of TMA pathophysiology that allowed drawing a TMA classification on the basis of risk factors and associated conditions. These findings may also result in the setting of targeted therapies to supply specific missing plasmatic proteins.</p></div>","PeriodicalId":100425,"journal":{"name":"EMC - Hématologie","volume":"2 1","pages":"Pages 14-34"},"PeriodicalIF":0.0,"publicationDate":"2005-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.emch.2004.12.002","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81411563","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: 10

Hémoglobines anormales rares

EMC - Hématologie Pub Date : 2004-11-01 DOI: 10.1016/j.emch.2004.08.004

B. Gulbis, F. Cotton, F. Vertongen

{"title":"Hémoglobines anormales rares","authors":"B. Gulbis, F. Cotton, F. Vertongen","doi":"10.1016/j.emch.2004.08.004","DOIUrl":"10.1016/j.emch.2004.08.004","url":null,"abstract":"<div><p>Hemoglobinopathies are genetic disorders of hemoglobin and are the most common inherited disorder in humans. The hemoglobin molecule is composed of four separate polypeptide chains, two alpha and two beta, as well as four iron-bearing heme groups that bind oxygen. The alpha chains are coded for by two similar genes on chromosome 16, the beta chains being coded by a single gene on chromosome 11. If most commonly, there is no structural or functional consequence, alteration of a single amino acid may have dramatic clinical consequences. This is the case with several unstable hemoglobins with high oxygen affinity or M hemoglobins. Depending on the molecular defect, unstable hemoglobins may induce hemolysis in vivo, hemoglobins with high affinity may induce compensatory erythrocytosis and hemoglobin M always cause pseudo cyanosis more or less visible. Generally, the molecular defect explains the pathology : unstable hemoglobins are frequently due to mutations in the heme pocket, hemoglobins with high affinity to mutations that alter the transition in the three-dimensional hemoglobin conformation that accompanies the removal of oxygen and M hemoglobins to mutations that stabilize the heme-iron in its oxidized form. Diagnosis of these rare hemoglobin variants is mandatory for adequate treatment, or in the case of hemoglobin M and hemoglobin with high affinity to prevent inappropriate investigations due to the mistaken impression that the patient has a cardiac, pulmonary or neoplastic disorder.</p></div>","PeriodicalId":100425,"journal":{"name":"EMC - Hématologie","volume":"1 4","pages":"Pages 106-114"},"PeriodicalIF":0.0,"publicationDate":"2004-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.emch.2004.08.004","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80294354","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

Index mots-clés 关键词索引

EMC - Hématologie Pub Date : 2004-11-01 DOI: 10.1016/S1638-6213(04)00022-9

引用次数: 0

Lymphomes folliculaires 淋巴癌卵泡

EMC - Hématologie Pub Date : 2004-11-01 DOI: 10.1016/j.emch.2004.06.001

C. Doyen , M. Delos , L. Michaux , A. Bosly

{"title":"Lymphomes folliculaires","authors":"C. Doyen , M. Delos , L. Michaux , A. Bosly","doi":"10.1016/j.emch.2004.06.001","DOIUrl":"https://doi.org/10.1016/j.emch.2004.06.001","url":null,"abstract":"<div><p>The incidence of follicular non-Hodgkin's lymphoma has increased dramatically in the past decades. Most of the affected subjects are aged persons, presenting with generalized adenopathy with bone marrow involvement. The course of the disease is often indolent, with a median survival of about 9 years, a continuous remitting and relapsing pattern, and a progressively shorter duration of remissions. Lymphoma itself is the main cause of death in these patients. Most of them have a histological grade 1 or 2; the actuarial risk of transformation into high grade lymphoma is 5 to 10 % per year. A new prognostic index, the <em>Follicular Lymphoma International Pronostic Index</em> (FLIPI), has been recently reported. In patients with low-tumour burden, therapy may be differed until progression, except in the localized stages, which may be cured by radiotherapy. Other therapies include alkylating agents, CVP and CHOP chemotherapies, purine nucleotide analogues, interferon and, more recently, the anti-CD20 monoclonal antibodies, which appear to be the beginning of a new therapeutic era. The indications for intensive treatments followed by hematopoietic stem cell transplantation have become more precise, generally resulting in prolonged remission. Moreover, allogeneic bone marrow transplant is curative for some patients. Clinical trials specifically designed to define the best strategy are necessary to determine whether the new therapeutic options are likely to modify the natural course of the disease and allow complete recovery.</p></div>","PeriodicalId":100425,"journal":{"name":"EMC - Hématologie","volume":"1 4","pages":"Pages 83-105"},"PeriodicalIF":0.0,"publicationDate":"2004-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.emch.2004.06.001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"92365002","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

Index auteurs

EMC - Hématologie Pub Date : 2004-11-01 DOI: 10.1016/S1638-6213(04)00021-7

引用次数: 0