Abstract:
BACKGROUND:Paroxysmal nocturnal hemoglobinuria (PNH) diagnostic guidelines recommend single-tube five- to six-color or two-tube four-color assays. PNH clones are detectable in only a fraction of patients at risk, and screening for new PNH cases can be complex and expensive. In this multicenter study, we have validated a simplified, one-tube two-color FLAER-based assay suitable for PNH screening. METHODS:Six laboratories received samples containing spiked PNH leukocyte clones to be analyzed in parallel with a common six-color cocktail (FLAER/CD24/CD45/CD64/CD15/CD14) and a simplified two-color mixture (FLAER/CD15), a shared calibration procedure, and a common analysis protocol. Replicate precision and sensitivity tests were performed on PNH patients, from undiluted to 1:10 000. Specificity tests were performed on normal donors to identify the possible sources of artifacts. RESULTS:The performance comparison between six-color and two-color assays showed an excellent agreement for granulocyte PNH clones. Dilution experiments showed an accurate detectability down to 0.01% sensitivity level for granulocyte PNH clones and to 1% for monocytes. Specificity experiments disclosed that basophils and platelets can contaminate the monocyte gate and generate false PNH events. CONCLUSIONS:A simplified two-color (FLAER/CD15) PNH screening test has been validated in a highly standardized multicenter study and proved feasible and effective in ongoing regional programs. Precision, sensitivity, and specificity of the simplified test for granulocytes were comparable to the more complex and expensive six-color assay and applicable for screening also in peripheral laboratories. The diagnostic confirmation of PNH should be always performed by a reference center using the established technique on all cell lineages.
journal_name
Eur J Haematoljournal_title
European journal of haematologyauthors
Gatti A,Del Vecchio L,Geuna M,Della Porta MG,Brando Bdoi
10.1111/ejh.12885subject
Has Abstractpub_date
2017-07-01 00:00:00pages
27-35issue
1eissn
0902-4441issn
1600-0609journal_volume
99pub_type
杂志文章,多中心研究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::33 patients with advanced refractory multiple myeloma received a combination of vincristine, cyclophosphamide, carmustine, melphalan and steroids (M-2 protocol). 20 of them had failed prior chemotherapy with alkylating agents and the remaining 13 patients had relapsed after a response to these drugs. An objective tumo...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1988.tb00816.x
更新日期:1988-02-01 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:OBJECTIVE:To determine the sensitivity and specificity of single-tube osmotic fragility (SOFT) and its different methods as screening test for thalassemia trait. METHODS:A cross-sectional study was conducted at Omair Sana Foundation. A total of 400 participants were included in the study. Three hundred were known thal...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12392
更新日期:2014-12-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1034/j.1600-0609.2003.00098.x
更新日期:2003-10-01 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
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journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1988.tb00182.x
更新日期:1988-04-01 00:00:00
abstract::Patients with ARF and haematological malignancy (excluding myeloma), presenting to a single unit over 10 years were analyzed to see if patients likely to benefit from intensive renal supportive therapy could be identified. 31 episodes of ARF were identified in 29 patients (mean age 51 +/- 2.9 yr): 19 were associated w...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1991.tb00133.x
更新日期:1991-08-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2011.01684.x
更新日期:2011-12-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2007.00952.x
更新日期:2007-11-01 00:00:00
abstract::Fourty-three episodes of fungaemia encountered from 1978 to 1991 in 43 patients with haematological malignancies are reviewed here to analyse the risk factors for death and to evaluate the efficacy of early antifungal therapy. Low serum cholinesterase and elevated serum blood urea nitrogen were significantly associate...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1993.tb00617.x
更新日期:1993-09-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Minimal residual disease (MRD) assessment by PCR in multiple myeloma (MM) has several shortcomings, including the lack of a suitable target. Kappa deleting element (KDE) rearrangements occur in virtually all Ig-lambda B-cell malignancies and in 1/3 of Ig-kappa are not affected by somatic hyper...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12000
更新日期:2012-10-01 00:00:00
abstract::Forty-six consecutive patients with acute lymphoblastic leukaemia (ALL), having a median age of 23 years (range 14 to 64), underwent induction and consolidation chemotherapy with weekly parenteral vincristine, adriamycin, l-asparaginase and daily oral prednisone (VAAP), followed by standard central nervous system (CNS...
journal_title:European journal of haematology
pub_type: 临床试验,杂志文章,随机对照试验
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更新日期:1992-08-01 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
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journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1034/j.1600-0609.2003.02846.x
更新日期:2003-01-01 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:OBJECTIVES:Granulocyte colony-stimulating factor-mobilized peripheral blood hematopoietic stem cell transplantation (HSCT) provides a valuable and increasingly used alternative to bone marrow transplantation (BMT). This retrospective study aimed at determining whether the stem cell source is predictive for outcome, rel...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2011.01692.x
更新日期:2011-12-01 00:00:00
abstract::In recent years, several direct-acting oral anticoagulants (DOAC) have become available for use in Europe and other regions in indications related to prophylaxis and treatment of venous and arterial thromboembolism. They include the oral direct thrombin inhibitor dabigatran etexilate (Pradaxa, Boehringer Ingelheim) an...
journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/ejh.12610
更新日期:2015-11-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1034/j.1600-0609.2002.02813.x
更新日期:2002-11-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章
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更新日期:2009-08-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章
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更新日期:2006-02-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1988.tb00209.x
更新日期:1988-10-01 00:00:00
abstract::Downregulation of cereblon (CRBN) gene expression is associated with resistance to the immunomodulatory drug lenalidomide and poor survival outcomes in multiple myeloma (MM) patients. However, the importance of CRBN gene expression in patients with myelodysplastic syndrome (MDS) and its impact on lenalidomide therapy ...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12457
更新日期:2015-07-01 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
abstract::The management of anticoagulant therapy (OAT) in patients with factor VII (FVII) deficiency is a very challenging clinical issue, as warfarin further reduces FVII levels, thus potentially increasing bleeding risk. On the other hand, the International Normalized Ratio test is misleading in such patients, as they do not...
journal_title:European journal of haematology
pub_type: 杂志文章,meta分析
doi:10.1111/ejh.13246
更新日期:2019-07-01 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
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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: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 isolated hereditary macrothrombocytopenia (CHMT) is a congenital form of macrothrombocytopenia that seems to be due to defective production secondary to a disturbance in megakaryocyte fragmentation. To better understand the pathogenesis of thrombopoiesis in this hereditary thrombocytopenic disorder, we determi...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1034/j.1600-0609.2000.90072.x
更新日期:2000-03-01 00:00:00
abstract::We evaluated the serum/plasma levels of cytokines [interleukin (IL)-6, vascular endothelial growth factor (VEGF), transforming growth factor (TGF)-beta2] and markers of coagulation, fibrinolysis, endothelial and platelet activation during the first 4 wk of treatment with the thalidomide analogue Actimid (CC-4047) in 1...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2004.00393.x
更新日期:2005-04-01 00:00:00