The hematology journal : the official journal of the European Haematology Association最新文献

{"title":"Evolution of microbial safety.","authors":"John A J Barbara","doi":"10.1038/sj.thj.6200426","DOIUrl":"https://doi.org/10.1038/sj.thj.6200426","url":null,"abstract":"","PeriodicalId":22486,"journal":{"name":"The hematology journal : the official journal of the European Haematology Association","volume":"5 Suppl 3 ","pages":"S69-73"},"PeriodicalIF":0.0,"publicationDate":"2004-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1038/sj.thj.6200426","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24561613","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":"Antiphospholipid syndrome.","authors":"Jef Arnout, Milosz Jankowski","doi":"10.1038/sj.thj.6200412","DOIUrl":"https://doi.org/10.1038/sj.thj.6200412","url":null,"abstract":"","PeriodicalId":22486,"journal":{"name":"The hematology journal : the official journal of the European Haematology Association","volume":"5 Suppl 3 ","pages":"S1-5"},"PeriodicalIF":0.0,"publicationDate":"2004-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24559892","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":"G-CSF induced progenitor mobilization in mice with PIGA- blood cells.","authors":"Bing Han,&nbsp;Jacqueline Unsinger,&nbsp;Fulu Liu,&nbsp;Dan C Link,&nbsp;Monica Bessler","doi":"10.1038/sj.thj.6200383","DOIUrl":"https://doi.org/10.1038/sj.thj.6200383","url":null,"abstract":"<p><strong>Objective: </strong>In patients with paroxysmal nocturnal hemoglobinuria (PNH) a proportion of blood cells are deficient in glycosyl phosphatidylinositol (GPI) anchored proteins due to a mutation in the PIGA gene. Previous studies showed that in PNH the majority of circulating early progenitor cells were normal but after G-CSF were mainly, of the PNH phenotype. This suggested that GPI-linked proteins contribute to the regulation of progenitor trafficking from bone marrow to peripheral blood.</p><p><strong>Methods: </strong>To test this hypothesis we studied progenitor cells in bone marrow, spleen, and peripheral blood in response to G-CSF in mice genetically engineered to have a proportion of blood cells deficient in GPI-linked proteins (LF mice).</p><p><strong>Results: </strong>In contrast to humans, LF and wild-type mice have comparable numbers of progenitor cells in bone marrow, spleen, and peripheral blood. Similarly, in LF mice the proportion of PIGA- progenitor cells in peripheral blood corresponds the proportion of PIGA- progenitor cells measured in bone marrow and spleen. After G-CSF the number of circulating progenitors significantly increased but the proportion of PIGA- cells remained the same in peripheral blood,bone marrow, and spleen.</p><p><strong>Conclusions: </strong>Our data indicate that under basal laboratory conditions the lack of GPI-linked protein does not cause a retention of progenitor cells in the bone marrow. This implies that the preferential circulation of normal progenitor cells in patients with PNH requires an additional component that most likely is provided by the altered microenvironment of the underlying bone marrow failure.</p>","PeriodicalId":22486,"journal":{"name":"The hematology journal : the official journal of the European Haematology Association","volume":"5 4","pages":"347-52"},"PeriodicalIF":0.0,"publicationDate":"2004-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40894516","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":"Chronic hepatitis C associated with Coombs-positive hemolytic anemia.","authors":"Ihab I Elhajj,&nbsp;Ala' I Sharara,&nbsp;Ali T Taher","doi":"10.1038/sj.thj.6200400","DOIUrl":"https://doi.org/10.1038/sj.thj.6200400","url":null,"abstract":"<p><p>Hepatitis C virus (HCV) is a recognized cause of significant extrahepatic disease. Induction of autoimmune hemolytic anemia (AIHA) has been reported, either during or after interferon (IFN) treatment of HCV infection. We herein report a 56-year-old patient with HCV infection who developed severe Coombs-positive AIHA in the absence of treatment with IFN. Prednisone therapy was initiated, but intravenous immunoglobulins were added because of persistent hemolysis. Clinical course was complicated by rapid deterioration and the development of Creutzfeldt-Jakob disease. Having discarded other possible causes of AIHA, we suggest a possible association between AIHA and infection by HCV.</p>","PeriodicalId":22486,"journal":{"name":"The hematology journal : the official journal of the European Haematology Association","volume":"5 4","pages":"364-6"},"PeriodicalIF":0.0,"publicationDate":"2004-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1038/sj.thj.6200400","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40894519","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":"Steady-state plasma lactoferrin levels in relation to infections and complications of sickle cell disease.","authors":"Iheanyi Okpala,&nbsp;Cynthia C Ugochukwu,&nbsp;Adel Tawil,&nbsp;Rachael Greaves","doi":"10.1038/sj.thj.6200372","DOIUrl":"https://doi.org/10.1038/sj.thj.6200372","url":null,"abstract":"","PeriodicalId":22486,"journal":{"name":"The hematology journal : the official journal of the European Haematology Association","volume":"5 4","pages":"371-2"},"PeriodicalIF":0.0,"publicationDate":"2004-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1038/sj.thj.6200372","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40894521","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":"Haemovigilance in the United Kingdom and Europe.","authors":"Dorothy Stainsby","doi":"10.1038/sj.thj.6200447","DOIUrl":"https://doi.org/10.1038/sj.thj.6200447","url":null,"abstract":"","PeriodicalId":22486,"journal":{"name":"The hematology journal : the official journal of the European Haematology Association","volume":"5 Suppl 3 ","pages":"S175-8"},"PeriodicalIF":0.0,"publicationDate":"2004-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1038/sj.thj.6200447","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24560367","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":"FLT3 tyrosine kinase as a target in acute leukemias.","authors":"James D Griffin","doi":"10.1038/sj.thj.6200450","DOIUrl":"https://doi.org/10.1038/sj.thj.6200450","url":null,"abstract":"","PeriodicalId":22486,"journal":{"name":"The hematology journal : the official journal of the European Haematology Association","volume":"5 Suppl 3 ","pages":"S188-90"},"PeriodicalIF":0.0,"publicationDate":"2004-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1038/sj.thj.6200450","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24560370","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":"Vaso-occlusion in sickle cell anemia: role of interactions between blood cells and endothelium.","authors":"Jacques E Elion,&nbsp;Manuel Brun,&nbsp;Marie-Heléne Odièvre,&nbsp;Claudine L Lapouméroulie,&nbsp;Rajagopal Krishnamoorthy","doi":"10.1038/sj.thj.6200452","DOIUrl":"https://doi.org/10.1038/sj.thj.6200452","url":null,"abstract":"","PeriodicalId":22486,"journal":{"name":"The hematology journal : the official journal of the European Haematology Association","volume":"5 Suppl 3 ","pages":"S195-8"},"PeriodicalIF":0.0,"publicationDate":"2004-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1038/sj.thj.6200452","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24560372","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":"Bone marrow histopathology and biological markers as specific clues to the differential diagnosis of essential thrombocythemia, polycythemia vera and prefibrotic or fibrotic agnogenic myeloid metaplasia.","authors":"Jan Jacques Michiels","doi":"10.1038/sj.thj.6200368","DOIUrl":"https://doi.org/10.1038/sj.thj.6200368","url":null,"abstract":"<p><p>Clinical, hematological and morphological peripheral blood and bone marrow characteristics, in particular, megakaryopoiesis and bone marrow cellularity, reveal diagnostic clues and pathognomonic features, which enable a clear-cut distinction between essential thrombocythemia (ET), polycythemia vera (PV) and prefibrotic and fibrotic agnogenic myeloid metaplasia (AMM). The characteristic increase of enlarged mature megakaryocytes with mature cytoplasm and multilobulated nuclei and their tendency to cluster in a normal or slightly increased cellular bone marrow represent the hallmark of ET. The characteristic increase and clustering of enlarged mature and pleiomorphic megakaryocytes with multilobulated nuclei and proliferation of erythropoiesis in a moderate to marked hypercellular bone marrow with hyperplasia of dilated sinuses are the specific diagnostic features of untreated PV. ET may precede PV for many years to more than one decade. Prefibrotic and fibrotic AMM appears to be a distinct dual proliferation of abnormal megakaryopoiesis and myelopoiesis. The histopathology of the bone marrow in prefibrotic and fibrotic AMM is dominated by atypical enlarged and immature megakaryocytes with cloud-like immature nuclei, which are not seen in ET and PV at diagnosis and during follow-up. Myelofibrosis is not a feature of ET at diagnosis and during long-term follow-up. Myelofibrosis, which is secondary to the megakaryocytic/granulocytic myeloproliferation, and extramedullary myeloid metaplasia constitute a prominent feature and usually progress more or less rapidly during the natural history of PV and AMM. Life expectancy is normal in ET, normal in the first and decreased in the second decade of follow-up in PV, but significantly shortened in thrombocythemia associated with prefibrotic AMM as well as in the various fibrotic stages of AMM. These clinical and pathological characteristics of the Ph-negative MPDs, by including bone marrow histopathology, enable a clear-cut distinction between ET, PV and prefibrotic and fibrotic AMM. The use of established and new biological markers of MPDs, like spontaneous EEC, PRV-1 gene expression etc, should be validated in large prospective multicenter studies of newly diagnosed and previously treated MPD patients using the proposed European clinical and pathological (ECP) criteria as the only gold standard available for the proper diagnosis and differential diagnosis of ET, PV and AMM.</p>","PeriodicalId":22486,"journal":{"name":"The hematology journal : the official journal of the European Haematology Association","volume":"5 2","pages":"93-102"},"PeriodicalIF":0.0,"publicationDate":"2004-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24438163","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":"Treatment options in chronic lymphocytic leukemia.","authors":"Emili Montserrat","doi":"10.1038/sj.thj.6200386","DOIUrl":"https://doi.org/10.1038/sj.thj.6200386","url":null,"abstract":"<p><p>Chronic lymphocytic leukemia (CLL) is the most common adult hematological malignancy in the Western world and predominantly affects the elderly. The disease encompasses a wide spectrum of clinical symptoms, which translate into variable prognosis and survival. The stratification of patients based on their clinical risk profile has been aided by the recognition of novel prognostic markers, for example, VH mutations and ZAP-70 expression, and this process is fundamental to assigning the most appropriate treatment strategy on an individual basis. Although CLL remains incurable with standard treatments, important progress in treatment has been made. The discovery of purine analogs such as fludarabine has led to significant improvements in remission rates and freedom from progression but, unfortunately, no significant prolongation in survival. With the success of newer therapeutic approaches, such as the monoclonal antibodies and stem cell transplantation, the focus of current therapy is on using these approaches in combination with fludarabine to produce high rates of molecular complete response, to eradicate minimal residual disease, and to lengthen survival. This paper provides an overview of CLL and discusses how recent therapeutic developments have changed the management of this form of leukemia.</p>","PeriodicalId":22486,"journal":{"name":"The hematology journal : the official journal of the European Haematology Association","volume":"5 Suppl 1 ","pages":"S2-9"},"PeriodicalIF":0.0,"publicationDate":"2004-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1038/sj.thj.6200386","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24464954","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}