Abstract:
:The aim of this study was to test the association between hematological/genetic factors and cerebral vasculopathy in children with sickle cell anemia (SCA). A group with cerebral vasculopathy (VASC) was composed of children who had stroke (n = 6), silent infarct (n = 11), or an abnormal transcranial Doppler (n = 5). Eighty-four patients had neither positive history of stroke or silent infarct, nor abnormal transcranial Doppler (NORM group). An intermediate group (COND; n = 15) was composed of SCA children with a conditional transcranial Doppler. Biological analyses were performed on samples obtained at steady state and before the beginning of any chronic treatment. The comparisons of the three groups demonstrated a protective effect of α-thalassemia against cerebral vasculopathy through its effects on hemoglobin and reticulocyte levels. Moreover, we observed higher frequency of G6PD deficiency in the VASC group compared with the other groups. Our study confirms the key role of α-thalassemia and G6PD status in the pathophysiology of cerebral vasculopathy in SCA children.
journal_name
Eur J Haematoljournal_title
European journal of haematologyauthors
Joly P,Garnier N,Kebaili K,Renoux C,Dony A,Cheikh N,Renard C,Ceraulo A,Cuzzubbo D,Pondarré C,Martin C,Pialoux V,Francina A,Bertrand Y,Connes Pdoi
10.1111/ejh.12607subject
Has Abstractpub_date
2016-04-01 00:00:00pages
404-8issue
4eissn
0902-4441issn
1600-0609journal_volume
96pub_type
杂志文章abstract:OBJECTIVES:The purpose of this study was to evaluate serum leptin levels in idiopathic thrombocytopenic purpura (ITP), in order to determine the influence of leptin on the pathogenesis of ITP. SUBJECTS AND METHODS:Forty-six untreated patients with chronic ITP were compared with 40 healthy people of similar age, sex an...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2004.00231.x
更新日期:2004-05-01 00:00:00
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::In 18 beta-thalassaemia families from the Ferrara area the incidence of an inherited low flavin mononucleotide (FMN)-dependent pyridoxine phosphate (PNP) oxidase activity, a sensitive indicator of red-cell FMN deficiency, is higher in related members in these families than in the unrelated spouses and controls subject...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1989.tb01225.x
更新日期:1989-04-01 00:00:00
abstract:OBJECTIVE:A retrospective study was performed to explore the effect of splenectomy on iron balance in thalassemia major (TM). METHODS:Twenty two TM patients treated with splenectomy were compared with a control group (non-splenectomized patients) matched for sex, age, pretransfusional Hb, chelation therapy, and durati...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12121
更新日期:2013-07-01 00:00:00
abstract::Bcl-2 expression, the number of apoptotic cells and the growth and differentiation of early bone marrow progenitor cells were studied in patients with confirmed diagnosis of acute myeloid leukaemia (AML). Bone marrow cells from normal individuals were used as controls. We observed an increased percentage of bcl-2-mono...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1999.tb01112.x
更新日期:1999-01-01 00:00:00
abstract::A number of small and lipophilic cations are able to reverse in vitro the resistance to anthracyclines and other natural products through their interaction with P-glycoprotein or P-gp. However, some modulators do not interact with P-gp. We have demonstrated in a previous a work, using confocal laser microspectrofluoro...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1998.tb01709.x
更新日期:1998-10-01 00:00:00
abstract::GM-CSF may induce pulmonary complications, such as dyspnea with temporary decreases in oxygen saturation described as first dose effect for higher dosages of intravenous rhGM-CSF. This study investigated possible pulmonary disturbances in adult de novo AML patients receiving yeast rhGM-CSF 24 h prior to chemotherapy u...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1997.tb01689.x
更新日期:1997-11-01 00:00:00
abstract:BACKGROUND:Immune checkpoint inhibitors (CPI) are widely used in modern oncology and have improved the prognosis of lung cancer, malignant melanoma, and other malignancies. Unlike cytotoxic chemotherapy, drugs such as nivolumab, pembrolizumab, and ipilimumab are associated with immune-related adverse effects. We recent...
journal_title:European journal of haematology
pub_type: 杂志文章,meta分析,评审
doi:10.1111/ejh.13187
更新日期:2019-02-01 00:00:00
abstract:OBJECTIVES:To investigate whether the glycoengineered type II anti-CD20 monoclonal antibody obinutuzumab (GA101) combined with the selective MDM2 antagonist idasanutlin (RG7388) offers superior efficacy to monotherapy in treating B-lymphoid malignancies in preclinical models. METHODS:The combined effect of obinutuzuma...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12756
更新日期:2016-11-01 00:00:00
abstract:OBJECTIVE:For transplantation of cord blood (CB) cells, it is important to select a CB sample that can reconstitute not only myelo-erythropoiesis but also lymphopoiesis in recipients. However, until now the reconstitution ability of CB samples has been assessed by colony forming unit-culture (CFU-C) assay or by simply ...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2007.00991.x
更新日期:2008-02-01 00:00:00
abstract::Cancer cells are characterized by having chromosomal abnormalities. The number of aberrations and the specific chromosomes affected are likely correlated with tumor progression. In this study, we analyzed the karyotype of 126 plasma cell leukemia (PCL) patients to identify the most frequently occurring imbalances and ...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2011.01629.x
更新日期:2011-07-01 00:00:00
abstract::In further studying the mechanism of action of IFN-alpha in HCL, we cultured the HCL cell line JOK-1 and the IFN-sensitive Burkitt cell line Daudi with and without IFN-alpha and investigated the changes in density of a number of surface antigens by use of mAb and flow cytometry analyses. During culture with IFN-alpha,...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1992.tb00586.x
更新日期:1992-03-01 00:00:00
abstract::In a prognostic univariate analysis of a series of 80 patients with idiopathic myelofibrosis the Hb-concentration, the platelet count and osteomyelosclerosis emerged as factors with prognostic significance. A Hb-concentration less than 10 g/dl was associated with a significantly shorter survival than a Hb-concentratio...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1990.tb00372.x
更新日期:1990-03-01 00:00:00
abstract:OBJECTIVES:An increased rate of platelet production is a possible cause of reduced antithrombotic response to once-daily aspirin. Markers of immature platelets (IPs), such as immature platelet count (IPC), immature platelet fraction (IPF), and mean platelet volume (MPV) might be useful for identifying patients who have...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.13109
更新日期:2018-09-01 00:00:00
abstract::Recent studies have greatly illuminated the genomic landscape of the myelodysplastic syndromes (MDS), and the pace of discovery is accelerating. The most common mutations found in MDS occur in genes involved in RNA splicing (including SF3B1, SRSF2, U2AF1, and ZRSR2) and epigenetic modification (including TET2, ASXL1, ...
journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/ejh.12515
更新日期:2015-07-01 00:00:00
abstract::Many hematopoietic cells undergo apoptosis when deprived of specific cytokines. Lipocalin 24p3, reported to be induced in hematopoietic cells by interleukin 3 (IL-3) depletion, induces hematopoietic cell apoptosis despite the presence of IL-3. As granulocyte colony stimulating factor (G-CSF) depletion also induces the...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1046/j.0902-4441.2003.00160.x
更新日期:2003-12-01 00:00:00
abstract:BACKGROUND:Haemorrhagic cystitis caused by BK virus (BKV) is a known complication of allogeneic haematopoietic cell transplantation (HCT) and is relatively common following HLA-haploidentical transplantation. Adoptive immunotransfer of virus-specific T cells from the donor is a promising therapeutic approach, although ...
journal_title:European journal of haematology
pub_type:
doi:10.1111/ejh.12848
更新日期:2017-06-01 00:00:00
abstract::Chronic myelomonocytic leukemia (CMML) is a myelodysplastic/myeloproliferative neoplasm, characterized by persistent monocytosis. Due to the lack of unique surface markers expressed by neoplastic monocytes and the frequent CD34-negative blast immunophenotype, the diagnostic value of flow cytometric immunophenotyping (...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12477
更新日期:2015-08-01 00:00:00
abstract::A case of hemoglobin H disease in combination with hemoglobin Constant Spring and a beta-globin chain variant is reported in a 3-yr-old Thai girl. On routine cellulose acetate electrophoresis, one abnormal band in addition to the hemoglobins A, A2, H, Bart's and Constant Spring was detected. The amount of this abnorma...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1034/j.1600-0609.2001.066005312.x
更新日期:2001-05-01 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::A panel of 14 monoclonal antibodies (McAb) against hematopoietic cell surface antigens was applied on mononuclear blood or bone marrow cells from 40 cases of acute leukemia in order to compare immunoenzymatic staining (IE) (alkaline phosphatase) of fixed cells with immunofluorescence staining (IF) of unfixed suspended...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1988.tb00884.x
更新日期:1988-08-01 00:00:00
abstract:OBJECTIVES:Hypocholesterolemia has been previously described in patients affected by thalassemia. In this study we retrospectively evaluated the cholesterol level in two groups of patients affected by either thalassemia major (TM) or thalassemia intermedia (TI), with the aim of establishing factors correlated to hypoch...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2008.01195.x
更新日期:2009-03-01 00:00:00
abstract::The development of an autoantibody to human Factor VIII is rare and presents many problems for diagnosis and treatment. We have seen several cases at our institution recently with widely heterogenous clinical and laboratory presentations. A wide range of treatment modalities were used in these cases with no gold stand...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1034/j.1600-0609.2001.00383.x
更新日期:2001-03-01 00:00:00
abstract::Targeting of cellular metabolism has emerged as a possible strategy in the treatment of human malignancies, and several experimental studies suggest that this therapeutic approach should also be considered in acute myeloid leukemia (AML). Clinical studies of metabolic intervention in AML patients with isocitrate dehyd...
journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/ejh.12690
更新日期:2016-03-01 00:00:00
abstract::Soluble tumour necrosis factor receptors (sTNF-Rs) play a role as modulators of the biological function of tumour necrosis factor-alpha (TNF-alpha) in an agonist/antagonist pattern. In various pathologic states the production and release of sTNF-Rs may mediate host response and determine the course and outcome of dise...
journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/j.1600-0609.1995.tb01618.x
更新日期:1995-01-01 00:00:00
abstract:OBJECTIVE:Diagnosing myeloid sarcoma remains challenging, and we aimed to provide clinicopathological features to facilitate diagnosis. METHOD:Clinicopathological data from 41 patients with de novo and 31 with secondary myeloid sarcoma were reviewed. RESULTS:Most de novo cases presented with isolated myeloid sarcoma ...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.13056
更新日期:2018-06-01 00:00:00
abstract::ECP (eosinophil cationic protein) was purified in high yield from the granules of human buffy coat eosinophils obtained from healthy individuals. The separation procedure included gel filtration on Sephadex G-75, ion-exchange chromatography on Bio Rex 70, and chelating chromatography on zinc-chelate Sepharose 6B. The ...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1988.tb00850.x
更新日期:1988-05-01 00:00:00
abstract::We have demonstrated that the intracellular processing of transferrin to effect iron removal involves two pathways, one sensitive to rotenone and the other not. We have also found that the effect of the rotenone is dependent on the transferrin concentration: iron uptake was suppressed with concentrations of transferri...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1990.tb00408.x
更新日期:1990-07-01 00:00:00
abstract::Platelet refractoriness arising from HLA immunization develops in 20-50% of multitransfused patients with hematologic malignancies. We retrospectively analyzed the efficiency of leukocyte-depleted blood components in preventing refractoriness in 140 adult patients with acute myeloid leukemia (AML), treated according t...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1994.tb01872.x
更新日期:1994-08-01 00:00:00
abstract::Karyotype analysis of bone marrow samples was performed in 20 cases of essential thrombocythemia (ET) at the time of diagnosis. Three patients had karyotype abnormalities at the time of diagnosis; trisomy 8, deletion of Y, and del(9)(q?) in each. The patient who had trisomy 8 at the time of diagnosis underwent myeloid...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1034/j.1600-0609.2002.01601.x
更新日期:2002-02-01 00:00:00