Abstract:
:Myelodysplastic syndromes (MDS) and the mixed myelodysplastic/myeloproliferative disorder juvenile myelomonocytic leukaemia (JMML) are rare haematopoietic stem cell diseases in children. While MDS-initiating events remain largely obscure, a growing body of clinical, genetic and laboratory evidence suggests that JMML is, at least in part, caused by aberrant signal transduction resulting from mutations of components of the RAS signalling pathway. To date, haematopoietic stem cell transplantation cures more than half of children diagnosed with MDS or JMML. Research on genetic conditions predisposing to MDS in young age, such as inherited syndromes with bone marrow failure, may present important insights into MDS pathogenesis.
journal_name
Br J Haematoljournal_title
British journal of haematologyauthors
Niemeyer CM,Kratz CPdoi
10.1111/j.1365-2141.2007.06958.xsubject
Has Abstractpub_date
2008-03-01 00:00:00pages
610-24issue
6eissn
0007-1048issn
1365-2141pii
BJH6958journal_volume
140pub_type
杂志文章,评审abstract::Mucormycosis has emerged as an increasingly important cause of morbidity and mortality in immunocompromised patients, but contemporary data in children are lacking. We conducted a nationwide multicentre study to investigate the characteristics of mucormycosis in children with haematological malignancies. The cohort in...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/bjh.16329
更新日期:2020-04-01 00:00:00
abstract::Alpha haemoglobin stabilising protein (AHSP) binds free alpha-globin chains and plays an important role in the protection of red cells, particularly during beta-thalassaemia. Murine ASHP was discovered as a GATA-1 target gene and human AHSP is directly regulated by GATA-1. More recently, AHSP was rediscovered as a hig...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.2006.06381.x
更新日期:2007-01-01 00:00:00
abstract::Sixty patients with haematological malignancies received a myeloablative regimen of total body irradiation, cyclophosphamide and fludarabine followed by a T-cell-depleted peripheral blood stem cell transplant from a human leucocyte antigen identical sibling. To improve donor immune function, 1 x 10(7) CD3+ cells/kg we...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.2005.05665.x
更新日期:2005-09-01 00:00:00
abstract::Red blood cells frozen by the low-glycerol fast-freezing technique were thawed, deglycerolized and resuspended in various media. The use of ACD-saline for resuspension markedly reduced in vitro haemolysis such that the red cells could be transfused up to 5 d after thawing. At this time the cells contained satisfactory...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1980.tb08718.x
更新日期:1980-04-01 00:00:00
abstract::The in vitro and in vivo activity of a deoxycytidine analogue, troxacitabine, alone or in combination with imatinib mesylate (IM), was evaluated against human chronic myeloid leukaemia (CML) cell lines both sensitive (KBM5 and KBM7) and resistant (KBM5-R and KBM7-R) to IM. These cell lines differ in their sensitivity ...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.2004.04831.x
更新日期:2004-03-01 00:00:00
abstract::MPLW515L/K and JAK2V617F can co-exist in myelofibrosis with myeloid metaplasia (MMM). The chronology of clonal emergence was studied in three such cases using serially stored bone marrow. At diagnosis, a major MPL515 mutant clone was accompanied by a minor JAK2V617F clone in all three instances. At 25 time points over...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.2006.06348.x
更新日期:2006-12-01 00:00:00
abstract::Changes in spleen size postallogeneic haematopoietic stem cell transplantation (HSCT) in patients with primary myelofibrosis have been poorly characterized. We analysed 10 patients with myelofibrosis and splenomegaly following a reduced-intensity allogeneic HSCT. All patients fully engrafted donor cells including five...
journal_title:British journal of haematology
pub_type: 杂志文章,多中心研究
doi:10.1111/j.1365-2141.2008.07010.x
更新日期:2008-04-01 00:00:00
abstract::Long term outcomes and mutations in patients with mantle cell lymphoma (MCL) who discontinued ibrutinib have not been described. Using deep targeted next generation sequencing, we performed somatic mutation profiling from 15 MCL patients (including 5 patients with paired samples; before and after progression on ibruti...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/bjh.15567
更新日期:2018-11-01 00:00:00
abstract::The importance of the subclassification of nodular sclerosing Hodgkin's disease (NSHD) according to the British National Lymphoma Investigation Group (BNLI) criteria, which had an independent prognostic value for 90 unselected patients diagnosed in our region in the period 1972-83, is still equivocal. Because survival...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.1997.d01-2010.x
更新日期:1997-02-01 00:00:00
abstract::Demand for anticoagulation management is increasing due to an expansion of clinical indications for therapy. One possible model of care to meet demand is patient self-management (PSM), beneficial to patients who need control over their condition. This study aimed to determine the cost and cost-effectiveness of PSM of ...
journal_title:British journal of haematology
pub_type: 杂志文章,随机对照试验
doi:10.1111/j.1365-2141.2006.06243.x
更新日期:2006-09-01 00:00:00
abstract::The role of apoptotic pathways in the development and function of the megakaryocyte lineage has generated renewed interest in recent years. This has been driven by the advent of BH3 mimetic drugs that target BCL2 family proteins to induce apoptosis in tumour cells: agents such as ABT-263 (navitoclax, which targets BCL...
journal_title:British journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/bjh.12757
更新日期:2014-04-01 00:00:00
abstract::Clinical trials with individual cytokines and extensive in vitro studies have provided the basis for the in vivo use of these molecules in combination. Animal models, with haemopoietic growth factors as well as preliminary studies in humans--as shown by our studies with the sequential use of IL-3 and GM-CSF in patient...
journal_title:British journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/j.1365-2141.1991.tb08131.x
更新日期:1991-10-01 00:00:00
abstract::Autologous transplantation has an established role in the treatment of lymphoproliferative disorders, but allogeneic transplantation remains controversial. In an attempt to reduce the high procedure-related mortality reported with allografting in lymphoma, we have used BEAM (BCNU, etoposide, cytarabine and melphalan),...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.2000.01879.x
更新日期:2000-03-01 00:00:00
abstract::The induction of anaemia and reticulocytosis by arylhydrazines was influenced by substituents on the benzene ring of phenylhydrazine. Arylhydrazines with ortho substituents, which would hinder the binding by haemoglobin of a ligand derived from the arylhydrazine, resulted in the least anaemia and reticulocytosis. Thes...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1976.tb01879.x
更新日期:1976-01-01 00:00:00
abstract::Among 80 hereditary spherocytosis (HS) kindreds studied using denaturing electrophoretic separation of solubilized eythrocyte membrane proteins, we recognized three prominent subsets: HS with isolated spectrin deficiency, HS with combined spectrin and ankyrin deficiency, and HS with band 3 deficiency These three subse...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.1997.1893005.x
更新日期:1997-07-01 00:00:00
abstract::The sensitivity of populations of human granulocyte precursors to factors with colony stimulating activity (CSA) was assessed in agar culture in vitro. The mean threshold of stimulation was estimated by analysis of dose-response curves of clone growth against concentration of CSA. This method of assessment has the adv...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1979.tb05892.x
更新日期:1979-04-01 00:00:00
abstract::We report two cases of acute myeloid leukaemia FAB classification M4Eo with high white cell counts at presentation, who developed acute respiratory failure with pulmonary infiltrates on chest radiograph soon after commencing conventional cytotoxic chemotherapy plus all-trans retinoic acid (ATRA). We suggest that in pa...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.2000.02043.x
更新日期:2000-06-01 00:00:00
abstract::Total knee replacement (TKR) is a safe treatment for alleviating pain and restoring physical function in end-stage arthropathy of the knee. First reports of TKR in haemophiliacs date back to the mid-1970s, however detailed information on long-term outcome is scarce. This study evaluated factors influencing the outcome...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.2009.07613.x
更新日期:2009-04-01 00:00:00
abstract::The in vitro haemostatic functions of fresh whole blood (FWB) are well preserved after cold storage. This study aimed to determine whether platelets derived from FWB and stored whole blood (SWB) contribute to clot formation in tissue injury after transfusion into coagulopathic rats with polytrauma/haemorrhage (T/H). T...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/bjh.14999
更新日期:2017-12-01 00:00:00
abstract::In this study, quiescent bone marrow-derived CD34+ erythroid burst-forming units (BFU-E) were found to be resistant to the inhibitory effects of tumour necrosis factor (TNF)-alpha and -beta as well as interferon (IFN)-alpha, -beta and -gamma, in contrast to those stimulated by a combination of erytrhropoietin (Epo) pl...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.2003.04566.x
更新日期:2003-10-01 00:00:00
abstract::Various levels of thrombin generation were induced by the infusion of a combination of factor Xa (F.Xa) and phosphatidylcholine/phosphatidylserine (PCPS) vesicles into normal dogs and non-human primates. In the dog, an immediate loss of von Willebrand factor antigen (vWF:Ag) with a progressive recovery to normal level...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1993.tb03219.x
更新日期:1993-12-01 00:00:00
abstract::Mantle cell lymphoma (MCL) is a heterogenic non-Hodgkin lymphoma entity, with a median survival of about 5 years. In 2008 we reported the early - based on the median observation time of 4 years - results of the Nordic Lymphoma Group MCL2 study of frontline intensive induction immunochemotherapy and autologous stem cel...
journal_title:British journal of haematology
pub_type: 临床试验,杂志文章
doi:10.1111/j.1365-2141.2012.09174.x
更新日期:2012-08-01 00:00:00
abstract::Systemic inflammation activates the tissue factor/factor VIIa complex (TF/FVIIa), leading to a procoagulant state, which may be enhanced by impairment of physiological anticoagulant pathways, such as the protein C system. Besides impaired protein C activation, resistance to activated protein C (APC) may occur. We stud...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.2006.06127.x
更新日期:2006-07-01 00:00:00
abstract::The Xba I polymorphic site in the factor VIII gene is present in the int22h-1 region which is found in two other copies (int22h-2 and int22h-3) distal to the gene. Previously the polymorphic status of the Xba I locus was studied by either Southern blot or PCR that amplified all three copies. Here we report the use of ...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.1999.01430.x
更新日期:1999-06-01 00:00:00
abstract::Because the phagocytic function of non-stimulated human polymorphonuclear granulocytes (PMN) is impaired after incubation with either polynuclear Fe(III) or Fe(II), we decided to study lipid peroxidation of PMN and monocytes by these iron complexes. Lipid peroxidation was assessed by measuring thiobarbituric acid reac...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1989.tb04328.x
更新日期:1989-08-01 00:00:00
abstract::There is limited information concerning the impact of physical activity and obesity on non-Hodgkin lymphoma (NHL) prognosis. We examined the associations between pre-diagnosis physical activity and body mass index (BMI) with survival in 238 diffuse large B-cell (DLBCL) and 175 follicular lymphoma cases, with follow-up...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/bjh.14702
更新日期:2017-08-01 00:00:00
abstract::Transient myeloproliferative disorder accompanied by Down's syndrome has been characterized as exhibiting self-limiting haematological abnormalities. We studied six patients suffering from this disorder in order to clarify the biological nature of their blast cells. Metaphases of leucocytes stimulated with phytohaemag...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1988.tb06187.x
更新日期:1988-02-01 00:00:00
abstract::Erythropoietin (EPO) and stem cell factor (SCF) are two important factors in human erythropoiesis. We have recently demonstrated that SCF and EPO synergistically activate mitogen-activated protein (MAP) kinase, thereby promoting growth of human erythroid colony-forming cells (ECFCs). In the present study, we have exam...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.2000.02145.x
更新日期:2000-07-01 00:00:00
abstract::Fetal haemoglobin levels have been measured prospectively in 135 autopsy cases of sudden, unexpected infant deaths (31 pre-term, 104 full term) using standard laboratory methods. These results have been compared with Hb F values from a normal control group of 570 living infants (145 pre-term, 425 full-term) with a pos...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1992.tb06440.x
更新日期:1992-10-01 00:00:00
abstract::Therapeutic options for treatment of recurrence of leukaemia after allogeneic bone marrow transplantation (BMT) are limited. A beneficial effect of donor lymphocyte infusions (DLI) has not previously been described in acute myeloid leukaemia (AML) relapse. We report a case of AML with t(8;21), relapsing 3 months after...
journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1046/j.1365-2141.1996.d01-1477.x
更新日期:1996-02-01 00:00:00