Bailliere's clinical haematology最新文献

{"title":"3 Pathogenesis and management of idiopathic myelofibrosis","authors":"BSc, MD, FRCP, FRCPath John T. Reilly (Consultant Haematologist)","doi":"10.1016/S0950-3536(98)80037-1","DOIUrl":"10.1016/S0950-3536(98)80037-1","url":null,"abstract":"<div><p>Idiopathic myelofibrosis is the least common and carries the worst prognosis of the chronic myeloproliferative disorders. The primary disease process is a clonal haematopoietic stem cell disorder which results in a chronic myeloproliferation and an atypical megakaryocyte hyperplasia. In contrast, the characteristic stromal proliferation is a reactive phenomenon, resulting from the inappropriate release of megakaryocyte/platelet-derived growth factors, including PDGF, TGF-β, bFGF and calmodulin. The median survival is approximately 4 years, although individual survival varies greatly. A variety of prognostic schema have been developed which enable the identification of high-risk patients, for whom bone marrow transplantation should be considered. Management for the majority of patients, however, is directed towards the alleviation of symptoms and improvement in quality of life. This review summarizes the recent advances in our understanding of the disease's pathogenesis and discusses the limited therapeutic options available to clinicians.</p></div>","PeriodicalId":77029,"journal":{"name":"Bailliere's clinical haematology","volume":"11 4","pages":"Pages 751-767"},"PeriodicalIF":0.0,"publicationDate":"1998-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3536(98)80037-1","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"21495848","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}

{"title":"4 Treatment of polycythaemia vera and essential thrombocythaemia","authors":"MD Ayalew Tefferi (Consultant, Associate Professor of Medicine) ,&nbsp;MD Murray N. Silverstein (Consultant, Professor of Medicine)","doi":"10.1016/S0950-3536(98)80038-3","DOIUrl":"10.1016/S0950-3536(98)80038-3","url":null,"abstract":"<div><p>The clinical course in both polycythaemia vera (PV) and essential thrombocythaemia (ET) is characterized by significant thrombohaemorrhagic complications and variable risk of disease transformation into myeloid metaplasia with myelofibrosis or acute myeloid leukaemia. Randomized studies have shown that the risk of thrombosis was significantly reduced in ET with the use of hydroxyurea (HU) and in PV with the use of chlorambucil or ³²P. However, the use of chlorambucil or ³²P has been associated with an increased risk of leukaemic transformation. Subsequently, other studies have suggested that both HU and pipobroman may be less leukaemogenic and as effective as chlorambucil and ³²P for preventing thrombosis in PV. However, the results from these prospective studies have raised concern that even HU and pipobroman may be associated with excess leukaemic events in both ET and PV. The recent introduction of anagrelide as a specific platelet-lowering agent, the demonstration of treatment efficacy with interferon-α, and the revived interest in using low-dose acetylsalicylic acid provide the opportunity to initiate prospective randomized studies incorporating these treatments.</p></div>","PeriodicalId":77029,"journal":{"name":"Bailliere's clinical haematology","volume":"11 4","pages":"Pages 769-785"},"PeriodicalIF":0.0,"publicationDate":"1998-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3536(98)80038-3","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"21496309","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}

{"title":"1 Diagnosis and classification of erythrocytoses and thrombocytoses","authors":"MD, FRCPath T.C. Pearson (Professor of Haematology)","doi":"10.1016/S0950-3536(98)80035-8","DOIUrl":"10.1016/S0950-3536(98)80035-8","url":null,"abstract":"<div><p>An erythrocytosis describes an increased peripheral blood packed cell volume (PCV) and is deemed to be absolute or apparent depending on whether or not the measured red cell mass (RCM) is above the reference range. This reference range must be related to the individual's height and weight to avoid erroneous interpretations using ml/kg total body weight expressions in obesity. Absolute erythrocytoses are divided into primary, where the erythropoietic compartment is intrinsically abnormal, secondary, where the erythropoietic compartment is normal but is responding to external pathological events leading to an increased erythropoietin drive, and idiopathic, where neither a primary nor a secondary erythrocytosis can be established. Both primary and secondary erythrocytoses have congenital and acquired forms. The only form of primary acquired erythrocytosis that has been defined is the clonal myeloproliferative disorder, polycythaemia vera (PV). Modified diagnostic markers for PV are proposed.</p><p>Thrombocytoses can be classified into primary, where megakaryopoiesis is intrinsically abnormal, secondary, where megakaryopoiesis is normal but increased platelet production is a reaction to some other unrelated pathology, and finally idiopathic. This latter new group would be used for patients not satisfying the criteria for primary or secondary thrombocytoses, if these were more precise and rigidly used than currently is the case. While theoretically congenital and acquired forms of primary and secondary thrombocytoses might exist, only one cause of secondary congenital thrombocytosis has been established, and primary congenital thrombocytosis has not yet been precisely defined. Primary (essential) thrombocythaemia (PT) is one of the forms of primary acquired thrombocytoses. The diagnostic criteria of PT traditionally involve the exclusion of secondary thrombocytoses and other myeloproliferative disorders but marrow histology could hold a key positive diagnostic role if objective histological features of PT were agreed.</p></div>","PeriodicalId":77029,"journal":{"name":"Bailliere's clinical haematology","volume":"11 4","pages":"Pages 695-720"},"PeriodicalIF":0.0,"publicationDate":"1998-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3536(98)80035-8","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"21495921","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}

{"title":"1 Thrombophilia: disorders predisposing to venous thromboembolism","authors":"MD, FRCP(C), FACP, FCCP Graham F. Pineo (Professor of Medicine and Oncology, Director, Thrombosis Research Unit) ,&nbsp;MB BS, MSc, FRCP(C), FACP, FCCP Russell D. Hull (Professor of Medicine, and Director, Thrombosis Research Unit)","doi":"10.1016/S0950-3536(98)80082-6","DOIUrl":"10.1016/S0950-3536(98)80082-6","url":null,"abstract":"<div><p>Venous thromboembolism continues to present a challenge to clinicians. Over the years, a number of risk factors which predispose to venous thromboembolism have been identified, and these risk factors are taken into account in the formulation of recommendations for the prevention and treatment of these disorders. In more recent years, there have been major advances in our understanding of congenital or acquired defects that predispose to thrombosis leading to these so-called acquired or inherited forms of thrombophilia. The list of acquired forms of thrombophilia now includes anti-thrombin, protein C, protein S, activated protein C resistance, the prothrombin 20210A mutant, homocysteinemia and a number of rare defects which either enhance coagulation or interfere with fibrinolysis. In spite of these advances, there are numerous families with thrombophilia in whom none of the known defects can be demonstrated. The challenge for the future is to discover some of these as yet unknown factors and to determine the most appropriate methods for the prevention and treatment of venous thromboembolism in susceptible individuals with thrombophilia.</p></div>","PeriodicalId":77029,"journal":{"name":"Bailliere's clinical haematology","volume":"11 3","pages":"Pages 525-540"},"PeriodicalIF":0.0,"publicationDate":"1998-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3536(98)80082-6","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"21199963","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}

{"title":"5 Heparin and low-molecular-weight heparin in the treatment of venous thromboembolism","authors":"MD, FRCP(C), FACP, FCCP Graham F. Pineo (Professor of Medicine and Oncology, Director, Thrombosis Research Unit) ,&nbsp;MB BS, MSc, FRCP(C), FACP, FCCP Russell D. Hull (Professor of Medicine and Director, Thrombosis Research Unit)","doi":"10.1016/S0950-3536(98)80086-3","DOIUrl":"10.1016/S0950-3536(98)80086-3","url":null,"abstract":"<div><p>Venous thromboembolism (deep vein thrombosis and pulmonary embolism) continues to constitute a major clinical challenge. Effective and safe prophylactic measures against venous thromboembolism are now available for most high risk patients. In spite of this, pulmonary embolism is responsible for approximately 150 000 to 200 000 deaths per year in the United States alone. Over the past 20 years, based on a number of high quality (Level I) clinical trials, patterns of practice with respect to the treatment of venous thromboembolism have changed dramatically. The standard treatment of venous thromboembolism has been the use of unfractionated heparin by continuous intravenous infusion, with laboratory monitoring using the activated partial thromboplastin time (APTT), with warfarin starting on day 1 or 2 and continued for 3 months. Unfractionated heparin has withstood the test of time and has been shown to be safe and effective in preventing recurrent venous thromboembolism and death in numerous clinical trials. The response of individual patients to heparin is highly variable, requiring frequent laboratory monitoring. Heparin has a number of other troublesome side effects including bleeding, heparin-induced thrombocytopenia and osteoporosis. The low-molecular-weight heparins have a number of advantages over unfractionated heparin. In particular, their increased bioavailability and prolonged half-life permit once daily subcutaneous injections and their predictable antithrombotic response based on body weight permits treatment without laboratory monitoring. Low-molecular-weight heparin in therapeutic doses causes less bleeding than unfractionated heparin and evidence is accumulating that the incidence of heparin-induced thrombocytopenia and osteoporosis are decreased as well. In individual clinical trials and meta-analyses, low-molecular-weight heparin treatment results in decreased recurrent thromboembolism, major bleeding and death when compared with unfractionated heparin in the treatment of deep vein thrombosis and pulmonary embolism. These agents have also been shown to be both effective and safe for the out-of-hospital treatment of venous thrombosis. Therefore, in many countries, low-molecular-weight heparin has replaced unfractionated heparin for the treatment of venous thromboembolism.</p></div>","PeriodicalId":77029,"journal":{"name":"Bailliere's clinical haematology","volume":"11 3","pages":"Pages 621-637"},"PeriodicalIF":0.0,"publicationDate":"1998-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3536(98)80086-3","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"21199967","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}

{"title":"8 Venous thromboembolism and cancer","authors":"BSc, MB, PhD, FRCS A.K. Kakkar (MRC Clinician Scientist Fellow and Senior Surgical Registrar),&nbsp;MD, CCST F. de Lorenzo (Hon. Lecturer),&nbsp;MD, FRCP(C), FACP, FCCP G.F. Pineo (Professor of Medicine and Oncology, Director, Thrombosis Research Unit) ,&nbsp;MA, MChir, MD, FRCS R.C.N. Williamson (Professor of Surgery)","doi":"10.1016/S0950-3536(98)80089-9","DOIUrl":"10.1016/S0950-3536(98)80089-9","url":null,"abstract":"<div><p>The association of thrombosis with malignant disease has been recognized for well over 100 years. Evidence from experimental and clinical studies indicates that the haemostatic system is involved in the growth, invasion and metastasis of tumours. Laboratory parameters of haemostasis are frequently deranged in patients with cancer and overt thrombosis is common spontaneously where it may be the first sign of malignancy or secondary to therapy. The mechanisms by which coagulation activation facilitates the malignant process remain to be completely elucidated, but it is clear that cells and proteins of the coagulation and fibrinolytic systems are involved at many steps in the processes of tumour growth and dissemination. The low-molecular-weight heparins with their well-proven safety and efficacy profiles offer unique modalities for the prevention and treatment of cancer-associated thrombosis. They may also play a role in overall mortality reduction in patients with malignant disease.</p></div>","PeriodicalId":77029,"journal":{"name":"Bailliere's clinical haematology","volume":"11 3","pages":"Pages 675-687"},"PeriodicalIF":0.0,"publicationDate":"1998-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3536(98)80089-9","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"21200581","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}

{"title":"3 Laboratory diagnosis of venous thromboembolism","authors":"MD, FRCPC Agnes Y.Y. Lee (Clinical Scholar),&nbsp;MD, FRCPC Jeffrey S. Ginsberg (Associate Professor, Director)","doi":"10.1016/S0950-3536(98)80084-X","DOIUrl":"10.1016/S0950-3536(98)80084-X","url":null,"abstract":"<div><p>Venous thromboembolism is a common medical condition in both out-patients and inpatients. Despite the development of non-invasive tests, the diagnosis of deep vein thrombosis and pulmonary embolism remains a clinical challenge. In an effort to improve diagnostic accuracy and to reduce the necessity of serial testing, laboratory markers of thrombin generation and fibrinolysis have been investigated as first-line screening tests. Although the majority of markers are elevated in acute thrombosis, D-dimer, a specific derivative of cross-linked fibrin, appears to have the most potential clinical utility. Accuracy studies and preliminary management trials suggest that rapid D-dimer enzyme-linked immunosorbent assays and the whole blood agglutination assay, SimpliRED D-dimer (Agen Biomedical, Brisbane, Australia), have strong potential as exclusionary tests in patients with suspected venous thrombosis.</p></div>","PeriodicalId":77029,"journal":{"name":"Bailliere's clinical haematology","volume":"11 3","pages":"Pages 587-604"},"PeriodicalIF":0.0,"publicationDate":"1998-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3536(98)80084-X","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"21199965","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}

{"title":"6 Oral anticoagulants for the treatment of venous thromboembolism","authors":"MD Jack E. Ansell (Professor of Medicine)","doi":"10.1016/S0950-3536(98)80087-5","DOIUrl":"10.1016/S0950-3536(98)80087-5","url":null,"abstract":"<div><p>Oral anticoagulation has been the mainstay of therapy for the long-term treatment of venous thromboembolism since the 1940s. The rationale for the use of oral anticoagulation is based on the results of both empirical clinical evidence and animal models of thrombosis in the 1950s and 1960s. Higher-quality studies emerged in the 1970s and 1980s demonstrating the benefit of initial heparinization for venous thromboembolism followed by long-term oral anticoagulation. Good clinical outcomes with oral anticoagulation are highly dependent on the quality of dose management. Excellent management is best achieved in a programme of focused and co-ordinated care, often referred to as an anticoagulation clinic. Such programmes achieve better outcomes at reduced costs because of fewer adverse events. New models of anticoagulation management are emerging with the development of point-of-care testing that enables patients to do their own prothrombin time monitoring and anticoagulation dose adjustment. These models have the potential to improve care further, to increase patient satisfaction and to reduce costs.</p></div>","PeriodicalId":77029,"journal":{"name":"Bailliere's clinical haematology","volume":"11 3","pages":"Pages 639-661"},"PeriodicalIF":0.0,"publicationDate":"1998-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3536(98)80087-5","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"21200579","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"2 Diagnostic management of venous thromboembolism","authors":"MD Roderik A. Kraaijenhagen (Registrar),&nbsp;Anthonie W.A. Lensing (Senior Registrar),&nbsp;MD Jos W. Wallis (Consultant in Radiology),&nbsp;MD Edwin J.R. van Beek (Senior Registrar),&nbsp;MD Jan W. ten Cate (Professor),&nbsp;MD Harry R. Büller (Professor)","doi":"10.1016/S0950-3536(98)80083-8","DOIUrl":"10.1016/S0950-3536(98)80083-8","url":null,"abstract":"<div><p>The accuracy of diagnostic methods for the diagnosis of deep vein thrombosis and pulmonary embolism in symptomatic patients is critically reviewed. In addition, the safety of withholding anticoagulant therapy from patients with suspected deep vein thrombosis or pulmonary embolism in whom the qualified diagnostic strategy was normal is evaluated by determining the frequency of venous thromboembolic complications during 3 months of follow-up. It is shown that the currently used available diagnostic techniques for deep vein thrombosis are all able to identify the majority of patients who indeed have venous thrombosis. However, as result of its accuracy and practical advantages, compression ultrasound is the test of choice in the evaluation of symptomatic patients. Patients with a normal test outcome should be re-tested to detect the small proportion of patients with proximally extending calf vein thrombosis. In the strategy of repeated diagnostic testing, impedance plethysmography could be used as an alternative to ultrasonography. To obtain a reduction in repeat tests various diagnostic strategies have been evaluated and it was shown that these strategies, using non-invasive tests, can be as accurate and safe as the invasive reference strategy. The safeties of the various strategies were very similar; however, important differences were observed with respect to the practical implementation of the various diagnostic strategies. Simplification of the repeated testing strategy by using a D-dimer assay and/or a clinical decision rule seems to be promising. The reference standard for the diagnosis of pulmonary embolism remains pulmonary angiography. Several strategies based on non-invasive diagnostic methods have been evaluated for their safety and complexability. Perfusion-ventilation lung scanning is the most thoroughly evaluated non-invasive technique so far. It seems safe to withhold anticoagulant therapy in patients suspected of pulmonary embolism with a normal perfusion lung scan result; however, further testing is needed in the case of a non-diagnostic perfusion-ventilation lung scan result. At this moment angiography is the method of choice in this category of patients. D-dimer assays, clinical decision rules and ultrasound examinations of the legs seem to have a high potential to limit the need for angiography. Also, spiral computerized tomography and magnetic resonance imaging are promising techniques, but their role in the diagnostic management of pulmonary embolism is still uncertain.</p></div>","PeriodicalId":77029,"journal":{"name":"Bailliere's clinical haematology","volume":"11 3","pages":"Pages 541-586"},"PeriodicalIF":0.0,"publicationDate":"1998-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3536(98)80083-8","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"21199964","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}

{"title":"Index","authors":"","doi":"10.1016/S0950-3536(98)80090-5","DOIUrl":"https://doi.org/10.1016/S0950-3536(98)80090-5","url":null,"abstract":"","PeriodicalId":77029,"journal":{"name":"Bailliere's clinical haematology","volume":"11 3","pages":"Pages 689-693"},"PeriodicalIF":0.0,"publicationDate":"1998-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3536(98)80090-5","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138277831","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}