Bailliere's clinical haematology最新文献_第4页

5 Inhibitory and activating human antiplatelet antibodies 抑制和激活人抗血小板抗体

Bailliere's clinical haematology Pub Date : 1998-06-01 DOI: 10.1016/S0950-3536(98)80053-X

PhD Hans Deckmyn (Professor in Chemistry), PhD Karen Vanhoorelbeke (Research Fellow), MD, PhD Kathelijne Peerlinck (Associate Professor in Medicine)

{"title":"5 Inhibitory and activating human antiplatelet antibodies","authors":"PhD Hans Deckmyn (Professor in Chemistry), PhD Karen Vanhoorelbeke (Research Fellow), MD, PhD Kathelijne Peerlinck (Associate Professor in Medicine)","doi":"10.1016/S0950-3536(98)80053-X","DOIUrl":"10.1016/S0950-3536(98)80053-X","url":null,"abstract":"<div><p>Platelets are essential for the maintenance of haemostasis and, on the other hand, play a pivotal role in the formation of a thrombus. It is clear that reduced platelet activity will result in a bleeding tendency, whereas stimulation of platelets can lead to thrombosis.</p><p>Human antiplatelet antibodies may not only result in thrombocytopenia, but they have also been found either to inhibit or activate platelets. Inhibition by antibodies of the function of different receptors on platelets, such as collagen receptors, the glycoprotein (GP) Ib/IX (acquired Bernard-Soulier syndrome) or the GPIIb/IIIa complex (acquired Glanzmann's thrombasthenia), results in a haemorrhagic disorder very similar to the situation where the respective receptors are absent.</p><p>On the other hand, reports have described a number of antibodies that activate platelets. The mechanism by which they do so varies and can involve interaction with the Fc receptor present on platelets, activation of the complement system or direct activation by binding to a signal-transducing antigen.</p><p>Although the presence of such antibodies is expected to aggravate the problems due to the frequently occurring immune thrombocytopenia, treatment of these patients essentially relies on classical immunosuppressive therapy. In the case of activating antibodies, antithrombotic measures, such as anticoagulants or antiplatelet agents, can be envisaged.</p></div>","PeriodicalId":77029,"journal":{"name":"Bailliere's clinical haematology","volume":"11 2","pages":"Pages 343-359"},"PeriodicalIF":0.0,"publicationDate":"1998-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3536(98)80053-X","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20970133","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: 11

12 Heparin-induced thrombocytopenia: diagnostic tests and biological mechanisms 肝素诱导的血小板减少症:诊断试验和生物学机制

Bailliere's clinical haematology Pub Date : 1998-06-01 DOI: 10.1016/S0950-3536(98)80060-7

PhD Jean Amiral (Scientific Director), MD, PhD Dominique Meyer (Professor)

引用次数: 12

6 Immune thrombocytopenic purpura in adults: clinical aspects 成人免疫性血小板减少性紫癜:临床方面

Bailliere's clinical haematology Pub Date : 1998-06-01 DOI: 10.1016/S0950-3536(98)80054-1

MD Shmuel Gillis (Senior Physician), MD Amiram Eldor (Head)

引用次数: 10

10 Thrombopoietin and its receptor: structure, function and role in the regulation of platelet production 血小板生成素及其受体:结构、功能及其在血小板生成调控中的作用

Bailliere's clinical haematology Pub Date : 1998-06-01 DOI: 10.1016/S0950-3536(98)80058-9

MD, PhD Albert E.G. Kr von dem Borne (Professor of Clinical Hematology and Immunohematology) , Claudia Folman (Doctorate Student) , Gabor E. Linthorst (Medical Student), MD Leendert Porcelijn (Head of the Laboratory of Platelet and Leukocyte Serology), Sonja van den Oudenrijn (Doctorate Student), MD, PhD Ellen van der Schoot (Head of the Immunocytology Laboratory), MD, PhD Masja de Haas (Senior Scientist)

{"title":"10 Thrombopoietin and its receptor: structure, function and role in the regulation of platelet production","authors":"MD, PhD Albert E.G. Kr von dem Borne (Professor of Clinical Hematology and Immunohematology) , Claudia Folman (Doctorate Student) , Gabor E. Linthorst (Medical Student), MD Leendert Porcelijn (Head of the Laboratory of Platelet and Leukocyte Serology), Sonja van den Oudenrijn (Doctorate Student), MD, PhD Ellen van der Schoot (Head of the Immunocytology Laboratory), MD, PhD Masja de Haas (Senior Scientist)","doi":"10.1016/S0950-3536(98)80058-9","DOIUrl":"10.1016/S0950-3536(98)80058-9","url":null,"abstract":"<div><p>Although thrombopoietin (Tpo) was already described in the early sixties, it took more than 30 years before it was cloned. It became possible after the recognition and cloning of its receptor, Mpl. In the past few years new information about Tpo and Mpl has accumulated rapidly. Structure, biosynthesis and tissue expression have been elucidated. The central role of Tpo in the regulation of megakaryocytopoiesis and platelet production has become clear, as has the way in which this is accomplished. Tpo appears to be an important growth factor for haematopoietic stem cells as well. Thus, it may become one of the most important factors in stem cell transplantation. Finally, the signal transduction mechanisms involved and the way in which Tpo affects platelets and their precursors functionally have been studied in detail.</p><p>The cloning and characterization of thrombopoietin and its receptor Mpl is one of the most important advances in the haematology of the nineties.</p></div>","PeriodicalId":77029,"journal":{"name":"Bailliere's clinical haematology","volume":"11 2","pages":"Pages 409-426"},"PeriodicalIF":0.0,"publicationDate":"1998-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3536(98)80058-9","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20969446","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: 11

15 The haemolytic-uraemic syndrome in childhood 儿童溶血性尿毒综合征

Bailliere's clinical haematology Pub Date : 1998-06-01 DOI: 10.1016/S0950-3536(98)80063-2

MD, PhD N.C.A.J. van de Kar (Resident in Pediatrics), MD, PhD L.A.H. Monnens (Professor in Pediatric Nephrology)

引用次数: 6

2 Acquired factor V inhibitors 2获得性因子V抑制剂

Bailliere's clinical haematology Pub Date : 1998-06-01 DOI: 10.1016/S0950-3536(98)80050-4

MD Paul Knöbl (Associate Professor), MD Klaus Lechner (Professor of Medicine and Head)

引用次数: 132

7 Immune thrombocytopenic purpura in pregnancy 妊娠期免疫性血小板减少性紫癜

Bailliere's clinical haematology Pub Date : 1998-06-01 DOI: 10.1016/S0950-3536(98)80055-3

MD, PhD G.C.M.L. Christiaens (Gynaecologist)

引用次数: 11

14 Thrombotic thrombocytopenic purpura: diagnosis, pathogenesis and modern therapy 14血栓性血小板减少性紫癜的诊断、发病机制及现代治疗

Bailliere's clinical haematology Pub Date : 1998-06-01 DOI: 10.1016/S0950-3536(98)80062-0

MD Amiram Eldor (Professor of Hematology)

{"title":"14 Thrombotic thrombocytopenic purpura: diagnosis, pathogenesis and modern therapy","authors":"MD Amiram Eldor (Professor of Hematology)","doi":"10.1016/S0950-3536(98)80062-0","DOIUrl":"10.1016/S0950-3536(98)80062-0","url":null,"abstract":"<div><p>Thrombotic thrombocytopenic purpura (TTP) is an uncommon multisystem disorder, sometimes associated with predisposing conditions such as pregnancy, cancer, exposure to certain drugs, bone marrow transplantation and HIV-1 infection. An abnormal interaction between the vascular endothelium and platelets which occurs in certain organs leads to thrombosis, endothelial proliferation, minimal inflammation and micro-angiopathic haemolysis. Recent studies suggest that endothelial cell perturbation and apoptosis caused by an as yet unknown plasma factor(s) may lead to the release of abnormal von Willebrand factor which facilitates the deposition of platelet microthrombi. Exchange transfusions of plasma or plasma-cryosupernatant remain the cornerstone of the treatment of TTP along with corticosteroids, platelet inhibitor drugs, vincristine and splenectomy. In most cases remissions can be attained, and cures are now common—although approximately one-half of the patients will relapse. While relapses are usually milder, they still carry a significant mortality and preventive therapies are not always effective.</p></div>","PeriodicalId":77029,"journal":{"name":"Bailliere's clinical haematology","volume":"11 2","pages":"Pages 475-495"},"PeriodicalIF":0.0,"publicationDate":"1998-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3536(98)80062-0","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20969450","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: 26

4 Immune-mediated thrombocytopenias: basic and immunological aspects 免疫介导的血小板减少:基础和免疫学方面

Bailliere's clinical haematology Pub Date : 1998-06-01 DOI: 10.1016/S0950-3536(98)80052-8

MD Leendert Porcelijn (Head of the Laboratory of Platelet and Leukocyte Serology, Department of Experimental Immunohematology), MD, PhD Albert E.G. Kr von dem Borne (Professor of Clinical Hematology and Immunohematology)

{"title":"4 Immune-mediated thrombocytopenias: basic and immunological aspects","authors":"MD Leendert Porcelijn (Head of the Laboratory of Platelet and Leukocyte Serology, Department of Experimental Immunohematology), MD, PhD Albert E.G. Kr von dem Borne (Professor of Clinical Hematology and Immunohematology)","doi":"10.1016/S0950-3536(98)80052-8","DOIUrl":"10.1016/S0950-3536(98)80052-8","url":null,"abstract":"<div><p>Acute idiopathic or autoimmune thrombocytopenic purpura (AITP) is a disorder found mainly in children, usually preceded by a viral infection, with a higher incidence in the autumn and winter. The platelet-specific autoantibodies in acute childhood AITP are more often of the IgM class. Chronic AITP occurs mostly in adults. The platelet immunofluorescence test (PIFT) detects platelet-specific autoantibodies with a sensitivity of 65–75%. The autoantibodies in chronic AITP are classified as IgG in 95%, IgM in 26% and IgA in 4% of cases. The antibodies are usually bound to platelets and are detectable as free circulating antibodies in about 40%. AITP in pregnancy may cause neonatal AITP by autoantibodies of the IgG class which pass the placenta barrier. The rare neonatal alloimmune thrombocytopenic purpura (NAITP) are caused by IgG alloantibodies against HPA-1a in 75–90%, HPA1b in 3–5%, HPA 3a in 4–5%, HPA5b in 6–19% and against private platelet antigens in 3%. To confirm the diagnosis of NAITP requires extensive serological testing of the child, and the parents have to be typed for the important platelet-specific antigens by PIFT, monoclonal antibody immobilisation of platelet antigens (MAIPA) and/or enzyme-linked immunosorbent assay (ELISA) techniques. Three mechanisms of drug-induced thrombocytopenias are described. Platelets of both the donor and the patient are destroyed in post-transfusion thrombocytopenic purpura (PTP) but PTP does not occur again if incompatible platelets are re-administered.</p></div>","PeriodicalId":77029,"journal":{"name":"Bailliere's clinical haematology","volume":"11 2","pages":"Pages 331-341"},"PeriodicalIF":0.0,"publicationDate":"1998-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3536(98)80052-8","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20970132","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: 19

1 The population genetics of the haemoglobinopathies 1血红蛋白病的群体遗传学

Bailliere's clinical haematology Pub Date : 1998-03-01 DOI: 10.1016/S0950-3536(98)80069-3

MRCPsych Jonathan Flint (Wellcome Trust Senior Clinical Fellow), PhD Rosalind M. Harding (University Research Lecturer, Faculty of Clinical Medicine), MA, DPhil Anthony J. Boyce (University Reader in Human Population Biology), MA, PhD John B. Clegg (Professor of Molecular Medicine)

{"title":"1 The population genetics of the haemoglobinopathies","authors":"MRCPsych Jonathan Flint (Wellcome Trust Senior Clinical Fellow), PhD Rosalind M. Harding (University Research Lecturer, Faculty of Clinical Medicine), MA, DPhil Anthony J. Boyce (University Reader in Human Population Biology), MA, PhD John B. Clegg (Professor of Molecular Medicine)","doi":"10.1016/S0950-3536(98)80069-3","DOIUrl":"10.1016/S0950-3536(98)80069-3","url":null,"abstract":"<div><p>The haemoglobinopathies are the commonest single-gene disorders known, almost certainly because of the protection they provide against malaria, as attested by a number of observations. The geographical distributions of malaria and haemoglobinopathies largely overlap, and microepidemiological surveys confirm the close relationship between them. For two of the commonest disorders, haemoglobin S and α<sup>+</sup>-thalassaemia, there is also good clinical evidence for protection against malaria morbidity. However, not all the evidence appears to support this view. In some parts of the world malaria and haemoglobinopathies are not, and never have been, coexistent. It is also difficult to explain why the majority of haemoglobinopathies appear to be recent mutations and are regionally specific. Here we argue that these apparent inconsistencies in the malaria hypothesis are the result of processes such as genetic drift and migration and of demographic changes that have occurred during the past 10 000 years. When these factors are taken into account, selection by malaria remains the force responsible for the prevalence of the haemoglobinopathies.</p></div>","PeriodicalId":77029,"journal":{"name":"Bailliere's clinical haematology","volume":"11 1","pages":"Pages 1-51"},"PeriodicalIF":0.0,"publicationDate":"1998-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3536(98)80069-3","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"21714469","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