Abstract:
BACKGROUND:This review describes the safety of deferoxamine (DFO), deferiprone (DFP), deferasirox (DFX) and combined therapy in young patients less than 25 yr of age with haemoglobinopathies. METHODS:Searches in electronic literature databases were performed. Studies reporting adverse events associated with iron chelation therapy were included. Study and reporting quality was assessed using AHRQ Risk of Bias Assessment Tool and McMaster Quality Assessment Scale of Harms. Prospective clinical studies were pooled in a random-effects meta-analysis of proportions. RESULTS:Safety data of 2040 patients from 34 studies were included. Ninety-two case reports of 246 patients were identified. DFX (937 patients) and DFP (667 patients) possess the largest published safety evidence. Fewer studies on combination regimens are available. Increased transaminases were seen in all regimens (3.9-31.3%) and gastrointestinal disorders with DFP and DFX (3.7-18.4% and 5.8-18.8%, respectively). Therapy discontinuations due to adverse events were low (0-4.1%). Reporting quality was selective and poor in most of the studies. CONCLUSION:Iron chelation therapy is generally safe in young patients, and published data correspond to summary of product characteristics. Each iron chelation regimen has its specific safety risks. DFO seems not to be associated with serious adverse effects in recommended doses. In DFP and DFX, rare, but serious, adverse reactions can occur. Data on combined therapy are scarce, but it seems equally safe compared to monotherapy.
journal_name
Eur J Haematoljournal_title
European journal of haematologyauthors
Botzenhardt S,Li N,Chan EW,Sing CW,Wong IC,Neubert Adoi
10.1111/ejh.12833subject
Has Abstractpub_date
2017-03-01 00:00:00pages
198-217issue
3eissn
0902-4441issn
1600-0609journal_volume
98pub_type
杂志文章,meta分析,评审abstract:BACKGROUND:Aberrant DNA methylation at CpG islands within promoters is increasingly recognised as a common event in human cancers and has been associated with the silencing of important tumour suppressor genes. Epigenetic therapy using hypomethylating agents has demonstrated clinical effectiveness; the drugs azacitidin...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12145
更新日期:2013-09-01 00:00:00
abstract:OBJECTIVE:Cell cycle arrest by FOXO transcription factors involves in transcriptional activation of p27(kip1), although the exact mechanism remains unclear. And it has been evidenced that reduced level of p27(kip1) which is frequently occurred in human cancers has been associated with poor prognosis. In this study, our...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2008.01077.x
更新日期:2008-08-01 00:00:00
abstract::The presence of CD25 and HC2 antigens in 66 different patients with acute myeloid leukemia (AML) was investigated. The expression of both antigens was observed in 32% of AML cells. Dual fluorescence staining experiments performed in 5 AML patient cells showed that CD25 and HC2 antigens were simultaneously expressed in...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1989.tb01467.x
更新日期:1989-05-01 00:00:00
abstract:BACKGROUND:Most results on the treatment of chronic myeloid leukaemia (CML) with imatinib were obtained from clinical trials that may differ from the routine practice. We report the results of treatment of consecutive patients with CML at ten major centres during 2000-2008. PATIENTS AND METHODS:Data reporting was retr...
journal_title:European journal of haematology
pub_type: 杂志文章,多中心研究
doi:10.1111/j.1600-0609.2011.01637.x
更新日期:2011-08-01 00:00:00
abstract::A 62-yr-old man with two healthy daughters was diagnosed with osteomyelofibrosis. To our surprise, a female XX-karyotype was observed in bone marrow and confirmed in PHA-stimulated T-lymphocytes from peripheral blood. Further molecular genetic investigation revealed a submicroscopic translocation between the short arm...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12555
更新日期:2015-09-01 00:00:00
abstract:OBJECTIVES:On the basis of two population-based registries, our study aims to calculate the real-world cost-effectiveness of rituximab maintenance compared with observation in relapsed or refractory follicular lymphoma patients who responded to second-line chemotherapy. METHODS:Data were obtained from the EORTC20981 t...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12264
更新日期:2014-01-01 00:00:00
abstract::An unusual case having IgM monoclonal gammopathy with clinical and pathologic features of multiple myeloma (MM) in association with neutrophilia and nephrotic syndrome is reported. The patient showed lytic bone lesions, decreased IgG and IgA levels, Bence-Jones proteinuria, nephrotic proteinuria with edema, and histol...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2007.00869.x
更新日期:2007-07-01 00:00:00
abstract::We retrospectively compared the antileukemic effects of chemotherapy alone and chemotherapy followed by modified donor lymphocyte infusion (DLI) in 82 patients with relapsed acute leukemia after haploidentical hematopoietic stem cell transplantation (HSCT) without in vitro T-cell depletion. We also investigated progno...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12168
更新日期:2013-10-01 00:00:00
abstract:OBJECTIVE:The mutational status of the immunoglobulin (Ig) V(H) gene in B-cell chronic lymphocytic leukaemia (B-CLL) identifies two subgroups of patients with significantly different outcomes. We investigated the association of ZAP-70 expression with IgVH mutational status in B-CLL by quantifying ZAP-70 mRNA, to evalua...
journal_title:European journal of haematology
pub_type: 临床试验,杂志文章
doi:10.1111/j.1600-0609.2005.00619.x
更新日期:2006-04-01 00:00:00
abstract::A baby girl born at 31 weeks gestation showed severe haemolytic anaemia and hyperbilirubinaemia which led to exchange transfusion within the first 12 hours of life. There was no blood group incompatibility between mother and child but there was a marked stomatocytosis of the baby's red blood cells. Family history reve...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1991.tb00554.x
更新日期:1991-07-01 00:00:00
abstract::Effective iron chelation therapy is an important part of treatment in patients with transfusion-dependent thalassaemia and lower-risk myelodysplastic syndromes (MDS). Key strategies for optimising iron chelation therapy include ensuring good adherence and preventing and managing adverse events (AEs). Good adherence to...
journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/ejh.13111
更新日期:2018-09-01 00:00:00
abstract:OBJECTIVE:Autologous stem cell transplantation (ASCT) has improved progression-free survival (PFS) and overall survival in eligible patients with newly diagnosed multiple myeloma (NDMM); however, relapse occurs. Maintenance therapy with lenalidomide (Len-Mt) extends survival and delays relapse and the subsequent initia...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.13497
更新日期:2020-11-01 00:00:00
abstract:OBJECTIVE:The exact fate of polymorphonuclear neutrophilic granulocytes (PMN; neutrophils) after their mobilization from the bone marrow is not known. It is believed that they, after a relatively short lifespan (1-3 d), become apoptotic and phagocytosed by macrophages. We have recently shown that transfused neutrophils...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2004.00263.x
更新日期:2004-08-01 00:00:00
abstract::In whole blood from splenectomized subjects (n = 8) in a steady state an increased number of reticulocytes was observed (14.0 +/- 7.8% versus 3.6 +/- 2.4% controls: p less than 0.05). Cell fractionation on density showed that reticulocytes, as in normals, are more or less confined to the least dense fraction; cell fra...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1988.tb00872.x
更新日期:1988-07-01 00:00:00
abstract:OBJECTIVES:Recent studies have indicated that patients who receive stem cell transplantation (SCT) and rituximab demonstrate an increased risk of developing hypogammaglobulinemia. Such hypogammaglobulinemia has been found to be due to delayed recovery of memory B cells with an abnormal cell marker expression and impair...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2008.01174.x
更新日期:2009-02-01 00:00:00
abstract::Marrow fibroblast colony formation was studied in patients with myeloproliferative disorders, using human serum or platelet-derived growth factor plus plasma-derived serum as growth-stimulating factors. Colony numbers negatively correlated with the patient's age, but were not different from those of controls. However,...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1990.tb00397.x
更新日期:1990-05-01 00:00:00
abstract:INTRODUCTION:Symptomology of AL amyloidosis can be vague, with a broad range of manifestations and potential etiologies. We sought to determine whether time from initial patient-reported symptom onset to diagnosis was associated with survival. METHODS:The Boston University Amyloidosis Patient Database was queried for ...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.13472
更新日期:2020-10-01 00:00:00
abstract::Agranulocytosis developed in a 63-year-old patient with myelodysplasia 6 weeks after commencing treatment with the oral iron chelator deferiprone (L1, 1,2-dimethyl-3-hydroxypyrid-4-one, CP20) at a daily dose of 79 mg/kg. This was the 3rd case of agranulocytosis (neutrophils 0 x 10(9)/l) in clinical trials of L1 at the...
journal_title:European journal of haematology
pub_type: 临床试验,杂志文章
doi:10.1111/j.1600-0609.1994.tb01323.x
更新日期:1994-11-01 00:00:00
abstract:BACKGROUND:The occurrence of a thrombotic event in congenital bleeding disorders has drawn considerable attention in recent years. Both patients with hemophilia and patients with von Willebrand disease and even those with rare coagulation disorders have been shown to present occasional thrombotic events. Little is know...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12129
更新日期:2013-08-01 00:00:00
abstract::We report here the first case with Diamond-Blackfan anemia (DBA) who responded to rituximab. The patient is an 8-yr-old Japanese girl with refractory DBA accompanied by complex congenital heart disease. She received two doses of rituximab, 375 mg/m(2)/wk. She became transfusion independent 6 months after the treatment...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2004.00394.x
更新日期:2005-05-01 00:00:00
abstract::In patients with myelodysplastic syndromes (MDS) and acute myeloid leukemia (AML), expression of the hematopoietic stem cell marker CD34 has been associated with a poorer prognosis. CD34 is usually analyzed by flow cytometry (FC), but may also be analyzed using immunohistochemistry (IH). The present study was undertak...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1996.tb01330.x
更新日期:1996-03-01 00:00:00
abstract::While cellular modulation in vitro of committed hematopoietic stem cell (HSC) growth has been known for some time, less is known about the effect of accessory cells (AC) on the growth of more immature HSC. We have examined the effect of peripheral blood (PB) AC on hematopoiesis by coculturing enriched PB CD34+ cells (...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1034/j.1600-0609.2002.01520.x
更新日期:2002-02-01 00:00:00
abstract:OBJECTIVE:Approximately 40% of idiopathic thrombotic thrombocytopenic purpura (TTP) patients will suffer an exacerbation (recurrence of TTP within 30 d after their last plasma exchange (PE) procedure), but there are no data to predict who is at greater risk. We studied the clinical utility of demographic and ADAMTS13 b...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2009.01331.x
更新日期:2009-12-01 00:00:00
abstract:BACKGROUND:Infections remain a leading cause of morbidity and mortality in patients with reduced immunity caused by haematological disease and chemotherapy-induced neutropenia. We evaluated the clinical and microbiological impact of discontinuing fluoroquinolone prophylaxis in these patients. METHODS:We analysed 154 a...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12345
更新日期:2014-10-01 00:00:00
abstract:INTRODUCTION/OBJECTIVES:Acute myeloid leukemia (AML) accounts for 90% of all cases of acute leukemia in adults. In Brazil, the mortality from myeloid leukemia is 1.74/100 000 men and 1.37/100 000 women. Our aim was to review and update guidelines of the Brazilian Society of Bone Marrow Transplantation on indications of...
journal_title:European journal of haematology
pub_type: 杂志文章,实务指引
doi:10.1111/ejh.12808
更新日期:2017-02-01 00:00:00
abstract:UNLABELLED:The role of growth hormone (GH) for maintaining normal erythropoiesis among non-GH-deficient elderly subjects is not known. OBJECTIVES:To determine relationships between the concentrations of serum insulin-like growth factor-1 (IGF-1) and it's carrier protein insulin-like growth factor binding protein 3 (IG...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2004.00374.x
更新日期:2005-02-01 00:00:00
abstract::It is well-known that uremic patients present prolonged bleeding times as a common complication. Factors responsible for this disorder have been extensively investigated. In order to elucidate the possible role of uremic middle molecules as responsible for the bleeding tendency observed in uremia, we have studied the ...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1987.tb00757.x
更新日期:1987-09-01 00:00:00
abstract:OBJECTIVES:To evaluate differences in clinical results according to age among patients with chronic myeloid leukemia (CML). METHODS:207 consecutive CML patients treated with imatinib frontline were revised, dividing them in young adults (>20 < 45 years) (YA), middle-aged adults (≥45 < 65 years) (MA) and elderly (≥65 y...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.13110
更新日期:2018-06-13 00:00:00
abstract::A 32-year-old male presented with isolated neutropenia 6 months after allogeneic bone marrow transplantation for CML from his HLA-matched brother. The presence of granulocyte-specific IgM and IgG antibodies in the patient's serum indicated an immune-mediated basis for the neutropenia. A variety of manoeuvres to suppre...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1988.tb00889.x
更新日期:1988-08-01 00:00:00
abstract:AIM:This review summarises the importance, recent progress and issues in measuring patient-reported outcomes (PROs) in haemophilia research. METHODS:A critical review of recent advances and trends in measuring haemophilia-related PROs was conducted, using current regulatory guidelines and methodological recommendation...
journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/ejh.12323
更新日期:2014-06-01 00:00:00