British Journal of Haematology
1365-2141
0007-1048
Anh Quốc
Cơ quản chủ quản: Wiley-Blackwell Publishing Ltd , WILEY
Lĩnh vực:
Hematology
Phân tích ảnh hưởng
Thông tin về tạp chí
The mission of the British Journal of Haematology (BJHaem) is to inform and educate its readership in all aspects of the clinical and laboratory practice of haematology, extending from its scientific basis, through translational research, to clinical trials and daily practice. The journal welcomes manuscripts in cutting edge science and new treatments including targeted therapy, molecular and gene therapy and immunotherapy. The journal also focuses on evidence-based medicine by publishing peer-reviewed Guidelines on various basic and clinical subjects resulting from committees formed by the British Society for Haematology.
Các bài báo tiêu biểu
Point‐of‐care testing in haemostasis Summary Point‐of‐care testing (POCT) in haematology has seen a significant increase in both the spectrum of tests available and the number of tests performed annually. POCT is frequently undertaken with the belief that this will reduce the turnaround time for results and so improve patient care. The most obvious example of POCT in haemostasis is the out‐of‐hospital monitoring of the International Normalized Ratio in patients receiving a vitamin K antagonist, such as warfarin. Other areas include the use of the Activated Clotting Time to monitor anticoagulation for patients on cardio‐pulmonary bypass, platelet function testing to identify patients with apparent aspirin or clopidogrel resistance and thrombelastography to guide blood product replacement during cardiac and hepatic surgery. In contrast to laboratory testing, POCT is frequently undertaken by untrained or semi‐trained individuals and in many cases is not subject to the same strict quality control programmes that exist in the central laboratory. Although external quality assessment programmes do exist for some POCT assays these are still relatively few. The use of POCT in haematology, particularly in the field of haemostasis, is likely to expand and it is important that systems are in place to ensure that the generated results are accurate and precise.
Tập 150 Số 5 - Trang 501-514 - 2010
Iron absorption by hypotransferrinaemic mice Summary Iron absorption rates by homozygous and wild‐type mice from a hypotransferrinaemic mouse colony were examined with in vivo tied‐off duodenal sengments and in vitro incubated duodenal fragments. Enhanced initial rates of mucosal uptake and carcass transfer by homozygotes, compared to wild‐types, were observed. The changes in vivo and in in vitro uptake kinetics resemble changes seen in iron deficient or hypoxic mice, suggesting that the liver iron loading shown by homozygotes is due to a failure of the normal mechanism for regulation of iron absorption. In vivo mucosal uptake and carcass transfer of radioiron showed an inverse correlation with liver non‐haem iron content in homozygous hypotransferrinaemic mice, suggesting that some degree of control of absorption, albeit at markedly reduced sensitivity, can operate in these mice. No correlation between haemoglobin level and iron absorption was observed in homozygous hypotransferrinaemic mice, suggesting that this regulator of iron absorption does not function in these mice. The precise pathogenic mechanism of the enhanced iron absorption in hypotransferrinaemia remains to be determined. Mucosal transferrin levels were found to parallel serum transferrin levels in homozygotes. heterozygotes and wild‐type mice. This supports previous suggestions that mucosal transferrin is derived from plasma transferrin and that the enhancement of iron absorption, by physiological mechanisms, does not require the presence of mucosal transferrin.
Tập 78 Số 4 - Trang 565-570 - 1991
Unexplained aplastic anaemia, immunodeficiency, and cerebellar hypoplasia (Hoyeraal‐Hreidarsson syndrome) due to mutations in the dyskeratosis congenita gene, <i>DKC1</i> Hoyeraal‐Hreidarsson (HH) syndrome is a multisystem disorder affecting boys characterized by aplastic anaemia (AA), immunodeficiency, microcephaly, cerebellar‐hypoplasia and growth retardation. Its pathogenesis is unknown. X‐linked dyskeratosis congenita (DC) is an inherited bone‐marrow‐failure syndrome characterized by skin pigmentation, nail dystrophy and leucoplakia which usually develop towards the end of the first decade of life. AA occurs in >90% of cases of DC. We speculated that mutations in the gene responsible for X‐linked DC (DKC1 ) may account for the HH syndrome, due to the phenotypic similarities between the disease in respect of AA and gender bias. We therefore analysed the DKC1 gene in two HH families. In one family a nucleotide change at position 361(A → G) in exon 5 was found in both affected brothers; in the other family a nucleotide change at position 146(C → T) in exon 3 was found in the affected boys. The finding of these two novel missense DKC1 mutations demonstrates that HH is a severe variant of DC. They also show that mutations in DKC1 can give rise to a very wide clinical spectrum of manifestations. Boys with unexplained AA or immunodeficiency should be tested for mutations in DKC1 even though they may lack diagnostic features of DC.
Tập 107 Số 2 - Trang 335-339 - 1999
Granulocyte colony‐stimulating factor (G‐CSF) induces the production of cytokines <i>in vivo</i> Granulocyte colony‐stimulating factor (G‐CSF) is a haematopoietic growth factor required for the proliferation and differentiation of haematopoietic precursors of neutrophil granulocytes and is now used to overcome congenital and acquired neutropenia. In addition to increasing the numbers of neutrophils in vivo and modulating neutrophil functions, G‐CSF may induce the production of cytokines such as tumour necrosis factor α (TNF‐α). In the present study, the plasma levels of granulocyte–macrophage colony‐stimulating factor (GM‐CSF) in six healthy volunteers given G‐CSF at 10 μg/kg once daily for 6 d were measured and found to be elevated. The elevated levels (P < 0·05) were detected on day 2, peaked on days 6–7 and returned to baseline on day 12. In vitro , G‐CSF did not enhance the secretion of TNF‐α and GM‐CSF from mononuclear cells, whole blood or endothelial cells. However, in the co‐presence of whole blood and endothelial cells, the secretion of TNF‐α was significantly enhanced by G‐CSF at low concentrations. The GM‐CSF secretion, however, was unaltered. G‐CSF pretreatment of whole blood suppressed lipopolysaccharide (LPS)‐induced secretion of TNF‐α and GM‐CSF in a dose‐dependent manner. These results together with our previous findings suggest that G‐CSF induces the production of TNF‐α and GM‐CSF in vivo , and that this production may be due to the co‐effects of endothelial cells and whole blood under the influence of G‐CSF through an as yet unknown network of cells and cytokines. Treatment of whole blood with G‐CSF suppresses LPS‐induced secretion of TNF‐α and GM‐CSF.
Tập 108 Số 4 - Trang 848-853 - 2000
The aetiology and management of Castleman disease at 50 years: translating pathophysiology to patient care Summary Fifty years ago, Dr Benjamin Castleman first described the unusual lymphoproliferative disorder that now bears his name. Over the subsequent decades, astute clinical and pathologic observations coupled with clever molecular biologic research have increased our understanding of the aetiology of Castleman disease (CD). This article proposes three broad CD variants based on both distinctive histopathology and clinical behaviour. The pivotal roles of infection with human herpesvirus 8 and interleukin‐6 production in the development of CD are emphasized. Finally, the natural history of CD and the myriad of therapeutic options are reviewed in the context of a unified model of CD pathophysiology, and continued areas of uncertainty are discussed.
Tập 129 Số 1 - Trang 3-17 - 2005
Transient and persistent expansions of large granular lymphocytes (LGL) and NK‐associated (NKa) cells: the Yorkshire Leukaemia Group study Summary. A survey of 870 different adult blood samples (primarily from patients with non‐haematological disorders) found that 269 (31%) had increased proportions (>25%) and/or absolute numbers (>1.0 × 109 /l) of morphologically‐defined large granular lymphocytes (LGL), and/or pheno‐typically‐defined NK‐associated (NKa) cells. Of these, 112 were re‐analysed at least 6 months after initial presentation and were classified as‘persistent’(92/112) or ‘transient’(20/112) according to whether or not the original abnormality was still present. Lymphocyte counts in most patients with persistent abnormalities were within normal limits (18/92) or slightly increased (68/92), with only six having a lymphocytosis exceeding 10.0 × 109 /l. With the exception of five persistent LGL expansions in which the granular lymphocytes did not express NKa determinants (designated LGL+ NKa‐), the remaining 87 cases could be phenotypically grouped according to their primary abnormality as CD8+ NKa+ (n = 33), CD4+ NKa+ (n=14), CD8dim+ NKa+ (n=7) or CD8− NKa+ (n=33). TCR genotypic studies in 58 patients showed that the 16 patients with rearranged TCR components were restricted to the CD8+ NKa+ group and that, in most of these, the CD8+ fraction showed abnormal relative CD16/CD56 expression. Persistent neutropenia (n=15) also appeared to be associated with primary abnormalities of CD8+ NKa+ cells (12/15). with 10 of these additionally showing rearranged TCR genes. In contrast, persistently increased CD8dim+ NKa+ and CD8– NKa+ components did not appear to phenotypically differ from their corresponding ‘counterparts’in normal bloods or in patients with transient LGL/NKa+ abnormalities. This survey has therefore established that persistent LGL/NKa+ abnormalities are considerably more common than suggested in published work, that a high proportion of patients with expanded CD8+ NKa+ components, with quite diverse clinical histories, show evidence of clonal lymphoid populations, and that the clonal nature of such disorders appears to be associated with abnormal NKa phenotypic patterns.
Tập 83 Số 3 - Trang 504-515 - 1993
Hodgkin's disease in the elderly: a population‐based study Summary. This study evaluated the incidence and outcome of Hodgkin's disease (HD) in older patients using a population‐based approach. In total, 102 patients (52 men, 50 women) aged ≥ 60 years presented in the Northern Health Region of England (population of 3·09 million) between 1 January 1991 and 31 December 1998 and were studied prospectively. The age‐specific incidence was 1·97/100 000 for those aged 60–69 years, and 2·18/100 000 for those aged 70 years or over. The median age of the cohort was 70 years (range 60–91) and the median follow up was 63 months (range 20–113). Out of 95 treated patients, 70 (74%) obtained complete or good partial (> 90% response) remissions. In the 60 to 69‐year‐old group, the disease‐specific survival at 5 years was 100% for those presenting with early stage disease and 52% for those with advanced stage disease. In patients aged >70 years the 5 year disease‐specific survival was 36% in patients with early stage and 14% for patients with advanced stage disease. The survival of patients with Epstein–Barr virus (EBV)‐positive tumours was significantly poorer than that of patients with EBV‐negative tumours (P = 0·007); median survival in the former group was 20 months versus undefined in the latter group. In total, 43 deaths were due to progressive HD and five were treatment‐related. This study defined the incidence of HD in our population and demonstrated that the prognosis of elderly patients, particularly those with advanced stage disease, has not improved concurrently with that of patients aged < 60 years old. Novel approaches to assessment and treatment are necessary.
Tập 119 Số 2 - Trang 432-440 - 2002
Impact of granulocyte colony‐stimulating factor (CSF) and granulocyte–macrophage CSF in patients with malignant lymphoma: a systematic review Summary. The granulopoiesis‐stimulating factors, granulocyte colony‐stimulating factor (G‐CSF) and granulocyte–macrophage colony‐stimulating factor (GM‐CSF), are used to prevent neutropenia and febrile neutropenia in patients with malignant lymphoma. The question as to whether G‐CSF/GM‐CSF improves dose‐intensity, tumour response and overall survival (OS) has not yet been answered. As the results from single studies are inconclusive, a systematic review was performed to determine the effectiveness of G‐CSF/GM‐CSF. Randomized controlled trials comparing prophylaxis with G‐CSF/GM‐CSF versus no prophylaxis in adult patients with malignant lymphoma undergoing conventional chemotherapy were included. Medical databases (Cochrane Library, Medline, Embase) and conference proceedings were searched. All authors were contacted to obtain missing data. We included 11 studies making a total of 1434 patients. Compared with no prophylaxis, G‐CSF/GM‐CSF significantly reduced the relative risk (RR) for neutropenia {RR 0·64 [95% confidence interval (CI) 0·55–0·75]} febrile neutropenia (RR 0·74 [95% CI 0·62–0·89]) and infection (RR 0·74 [95% CI 0·64–0·85]). G‐CSF/GM‐CSF did not decrease infection‐related mortality (RR 2·07 [95% CI 0·81–5·34]), improve complete remission (CR) (RR 1·06 [95% CI 0·96–1·16]) or OS (HR 0·98 [95% CI 0·81–1·18]). In conclusion, G‐CSF/GM‐CSF given during conventional chemotherapy in malignant lymphoma patients reduced the RR of neutropenia, febrile neutropenia and infection. However, there is no evidence that G‐CSF/GM‐CSF improved CR and OS in this clinical setting.
Tập 122 Số 3 - Trang 413-423 - 2003
Causes of Macroplania of Erythrocytes in Diseases of the Liver and Biliary Tract with Special Reference to Leptocytosis Summary In diseases of the liver or biliary tract increased surface area (macroplania) is related either to macrocytosis or to leptocytosis. In a study on 20 patients with macroplania associated with leptocytosis, but without target cells, the following observations were made.
There was a positive correlation between changes in MCD and serum bilirubin. There was a negative correlation between MCD and osmotic fragility. There was a positive correlation between MCD and mean amount of cholesterol per erythrocyte and per μm2 of erythrocyte surface area, but there was no correlation between mean amount of cholesterol per erythrocyte and serum concentration of free or total cholesterol. The osmotic fragility of normal erythrocytes introduced into the circulation of patients with leptocytosis became similar to the (diminished) osmotic fragility of the patients' own erythrocytes.
The order in which the changes occur is probably as follows: changes in the peripheral circulation→increase in the mean amount of cholesterol per erythrocyte and per unit of erythrocyte surface area→increase in mean erythrocyte surface area while the MCV remains normal→increase of MCD and diminution of MCT→diminution of osmotic fragility.
Tập 19 Số 2 - Trang 223-235 - 1970