Abstract:
OBJECTIVES:Beta thalassaemia major (β-TM) and sickle-cell disease (SCD) are severe haemogobinopathies requiring life-lasting, advanced medical management. In the Mediterranean region, both conditions occur with high frequency. We assessed the efficacy of the National Program for the Prevention of Haemoglobinopathies in Greece during the last 30 yrs. METHODS:Data of affected births between 01/01/1980 and 31/12/2009 were collected in a nationwide scale, and expected vs. observed rates of new births were calculated and compared. In a subpopulation of affected births of Greek origin, the causes for occurrence of the new affected birth were also collected and analysed. RESULTS:Overall, the reduction in new cases was 81.1% and 84.6% for β-TM and SCD, respectively. For β-TM, a constant declining trend was recorded over the 30-yr period, whereas for SCD, a transient reversal was observed in the mid-1990s probably due to the significant influx of immigrants of African origin. Programme failure was 2.2 times more common among new β-TM births of Greek origin compared to new SCD cases (P < 0.001). Unawareness and parental choice were more frequent in SCD compared to β-TM (unawareness: OR = 1.4, P = 0.05, parental choice: OR = 1.9, P = 0.01). The main cause for programme failure was carrier misidentification and incorrect genetic advice for β-TM and SCD, respectively. CONCLUSIONS:The β-TM and SCD prevention programme in Greece has significantly reduced the numbers of new affected births. The outcomes could be optimised in groups of non-Greek origin, in carrier identification and by offering specialised genetic counselling.
journal_name
Eur J Haematoljournal_title
European journal of haematologyauthors
Ladis V,Karagiorga-Lagana M,Tsatra I,Chouliaras Gdoi
10.1111/ejh.12076subject
Has Abstractpub_date
2013-04-01 00:00:00pages
313-22issue
4eissn
0902-4441issn
1600-0609journal_volume
90pub_type
历史文章,杂志文章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::Myeloproliferative neoplasms associated with FIP1L1-PDGFR rearrangements represent a rare subset of myeloid and lymphoid malignancies, characterised by the presence of eosinophilia and abnormalities of PDGFRA, PDGFRB or FGFR1 genes. The fusion product of such genes is a tyrosine kinase oncoprotein sensitive to imatini...
journal_title:European journal of haematology
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journal_title:European journal of haematology
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doi:10.1111/j.1600-0609.2005.00504.x
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abstract:OBJECTIVES:Combined high-dose Interferon-alpha and psoralen plus ultraviolet A irradiation (PUVA) have been reported to be effective in the treatment of early mycosis fungoides (MF); however, our study is the first controlled prospective study in the literature exploring the activity and tolerability of the combination...
journal_title:European journal of haematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1111/j.1600-0609.2005.00497.x
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abstract::Neutrophil gelatinase-associated lipocalin (NGAL) is a siderphore binding molecule present in the specific granules of neutrophils and induced in a variety of epithelial cells during inflammation. Its mouse orthologue, 24p3, is also an acute phase protein synthesized in the liver and adipose tissue during inflammation...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2005.00511.x
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abstract::Over the last two decades, the role of microRNAs has been extensively investigated, and it has become clear that these small non-coding RNAs play an essential role in several biological processes including erythropoiesis and that their dysregulation is associated with pathologies. Recent technical innovations have con...
journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/j.1600-0609.2012.01774.x
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abstract::The development of an unusual case of primary pleural effusion in a 90-year-old human immunodeficiency virus (HIV)-negative Japanese woman with no identifiable tumor mass has been described. Pleural effusion specimens contained large diffuse lymphoma cells, with the phenotype and genotype of a B-cell lineage (positive...
journal_title:European journal of haematology
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journal_title:European journal of haematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1111/ejh.13318
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journal_title:European journal of haematology
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doi:10.1111/j.1600-0609.2004.00394.x
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journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/j.1600-0609.1992.tb00043.x
更新日期:1992-10-01 00:00:00
abstract::Recombinant human erythropoietin (rhEpo) is a mainstay in the treatment of anaemia, primarily in renal failure. Because the half-life of circulating rhEpo is relatively short (4-8 h), the drug is usually administered 2-3 times weekly. Recently, a novel erythropoiesis-stimulating protein (NESP) with a longer half-life ...
journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1034/j.1600-0609.2002.02813.x
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abstract::In addition to BCR, various rare fusion partners for the ABL1 gene have been reported in leukemia. We have identified the fusion gene SNX2-ABL1 in a pediatric case of acute lymphoblastic leukemia (ALL), which has only once previously been reported in an adult patient. Cytogenetic analysis detected this fusion gene ari...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12234
更新日期:2014-03-01 00:00:00
abstract::A consecutive material of 111 untreated patients with non-Hodgkin's lymphoma was studied with respect to fraction of S-phase cells in blood mononuclear cells in relation to presence of monoclonal B cells in blood (MBCB). Fraction of S-phase cells was determined by flow cytometry and estimation of MBCB was performed by...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1989.tb01221.x
更新日期:1989-04-01 00:00:00
abstract::Human red blood cells (RBC) are heterogeneous with respect to their size; the physiological significance of this heterogeneity has not yet been fully elucidated. To further investigate this problem, some characteristics of human RBC fractionated according to their mean corpuscular volume (MCV) by counterflow centrifug...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1992.tb00570.x
更新日期:1992-02-01 00:00:00
abstract::It is well-documented that iron chelation by desferrioxamine protects/improves the cardiac function in blood transfusion-dependent children suffering from beta-thalassaemia. In patients who do not become dependent upon blood transfusion until adulthood (ANT-patients), iron chelation by desferrioxamine may affect the c...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1997.tb00981.x
更新日期:1997-10-01 00:00:00
abstract:OBJECTIVES:Central retinal vein occlusion (CRVO) and central retinal artery occlusion (CRAO) are common disorders associated with risk factors for atherosclerosis. Protein Z is a cofactor for the inactivation of activated factor X (Xa) by the protein Z dependent protease inhibitor. Protein Z deficiency was recently lin...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2005.00529.x
更新日期:2005-11-01 00:00:00
abstract:OBJECTIVES:Imatinib (Glivec, STI571) has been successfully used in patients with chronic myelogenous leukemia and acute lymphoblastic leukemia with the Philadelphia chromosome (Ph). We used imatinib interim therapy for four consecutive patients with newly diagnosed Ph+ acute myeloid leukemia (AML). We monitored the pat...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2007.00890.x
更新日期:2007-08-01 00:00:00
abstract::During the period 1978-1982 in the three northernmost countries of Sweden all 143 patients with a registered diagnosis of chronic lymphocytic leukemia (CLL) were retrospectively analyzed. After re-evaluation, 122 cases remained in the study. The mean age was 71 years and the male/female ratio was 2.2:1. Sixty-one pati...
journal_title:European journal of haematology
pub_type: 杂志文章,多中心研究
doi:10.1111/j.1600-0609.1994.tb01295.x
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abstract:OBJECTIVES:Northern Norway consists of a multiethnic population of Sámi and non-Sámi. We evaluated iron status in these two groups with respect to gender, age and residence. METHODS:In 2002-2004, a cross-sectional study of health and living conditions in areas with both Sámi and Norwegian populations, SAMINOR, was per...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2007.00929.x
更新日期:2007-11-01 00:00:00
abstract::We previously reported an increased risk of monoclonal gammopathy of undetermined significance (MGUS) in first-degree relatives of MGUS and multiple myeloma patients. Here, we examine whether primary cytogenetic categories of myeloma differ between patients with and without a family history of MGUS or myeloma. We stud...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12133
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journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1991.tb01575.x
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journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.13468
更新日期:2020-10-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12018
更新日期:2013-04-01 00:00:00
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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::The oncogenes c-myc and c-ras are known to elicit a cooperative tumorigenicity. In this study we investigated their role in the pathogenesis of Hodgkin's disease. The expression of these oncogenes was determined in Hodgkin's disease patients by avidin-biotin peroxidase complex immunohistochemical staining and was comp...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1995.tb00247.x
更新日期:1995-09-01 00:00:00
abstract:OBJECTIVES:In children with sickle cell disease (SCD), chronic transfusion to maintain haemoglobin S (HbS) below 30% markedly decreases both the risk of a first stroke when transcranial Doppler (TCD) ultrasonography shows abnormal cerebral blood flow velocities and the risk of recurrent stroke. Maintaining HbS below 30...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2009.01379.x
更新日期:2010-03-01 00:00:00
abstract::Antigenic concentration of total kininogen, kinin liberated in vitro, and the antigenic concentration of high molecular weight (HMW) kininogen was measured in 58 different samples of cord blood plasma and in plasma samples from 67 healthy blood donors. Total kininogen and kinin concentration in cord blood plasma was m...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1991.tb01536.x
更新日期:1991-05-01 00:00:00
abstract:INTRODUCTION:Tumor cells of primary cutaneous T-cell lymphomas are able to adopt a regulatory T-cell phenotype in vitro. The significance of this finding in vivo is matter of debate. METHODS:We stained five cases with transformed mycosis fungoides (MF) with an antibody against FOXP3, which is a sensitive and specific ...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2006.00809.x
更新日期:2007-03-01 00:00:00
abstract:OBJECTIVE:The extent of disease in patients with multiple myeloma or related conditions may be difficult to assess. In previous small studies, both FDG-PET (PET) and Tc-99m sestamibi scans (MIBI) have identified sites of occult disease in myeloma. METHODS:We reviewed the results for patients at our institution who hav...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1046/j.0902-4441.2004.00177.x
更新日期:2004-01-01 00:00:00
abstract::Involvement of the central nervous system (CNS) in multiple myeloma (MM) is very uncommon; it has been observed in approximately 1% of the MM patients. This review summarizes the clinical and laboratory characteristics and treatment modalities of 109 patients with CNS myelomatosis (CNS MM) reported in the literature. ...
journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/j.1600-0609.2007.00956.x
更新日期:2008-01-01 00:00:00