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 abnormal band movement towards more anodic to the hemoglobin A was 35.7%. DNA analysis of the alpha-globin gene cluster by polymerase chain reaction (PCR) revealed a combination of defects caused by the SEA-type alpha-thalassemia 1 and the alpha-Constant Spring gene. Analysis of beta-globin gene by PCR and DNA sequencing also detected the heterozygosity for the GGC-GAC mutation at codon 56, leading to a substitution of aspartic acid for glycine resulting in the hemoglobin J Bangkok. The hematologic data of this unusual case of hemoglobin H disease are presented and compared with two compound heterozygotes for hemoglobin J Bangkok and alpha-thalassemia 1 found in the patient's father and grandfather. A simple DNA assay based on an allele-specific PCR for rapid diagnosis of the hemoglobin J Bangkok is also described.
journal_name
Eur J Haematoljournal_title
European journal of haematologyauthors
Fucharoen S,Ayukarn K,Sanchaisuriya K,Fucharoen Gdoi
10.1034/j.1600-0609.2001.066005312.xsubject
Has Abstractpub_date
2001-05-01 00:00:00pages
312-6issue
5eissn
0902-4441issn
1600-0609pii
ejh419journal_volume
66pub_type
杂志文章abstract::The heat production rate in peripheral blood lymphocytes was evaluated by direct calorimetry in 76 untreated adults with non-Hodgkin's lymphoma (NHL). Elevated values were recorded for 20 out of 54 patients with lymphomas of high or intermediate malignancy grade (37%) and for 1 out of 22 patients (5%) with low grade l...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1990.tb00469.x
更新日期:1990-11-01 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::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:OBJECTIVE:We report an extension study of patients with essential thrombocythaemia (ET) in the Hungarian Myeloproliferative Neoplasm (HUMYPRON) Registry, which demonstrated that over 6 years anagrelide significantly decreased the number of patients experiencing minor arterial and minor venous thrombotic events (TEs) vs...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.13459
更新日期:2020-10-01 00:00:00
abstract:OBJECTIVES:Internal tandem duplications (ITDs) of the fms-like tyrosine kinase 3 ( FLT3) gene occur in 13-35% of patients with acute myeloid leukemia (AML). FLT3-ITD is associated with poor clinical outcome and is an indication for allogeneic stem cell transplantation (allo-SCT). METHODS:To investigate FLT3-ITD length...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2012.01785.x
更新日期:2012-07-01 00:00:00
abstract::Ten patients, who received cadaveric kidneys, were followed for 24 wk with serial measurements of serum erythropoietin (S-Epo), transferrin receptor (S-TfR) and iron variables. The mean pretransplant creatinine clearance was 8.2 (range 0-22) ml/min and the mean haemoglobin (Hb) level was 99 +/- 18.6 (range 66-124) g/l...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1997.tb00943.x
更新日期:1997-03-01 00:00:00
abstract::The prevalence of obesity is increasing and progressively influencing physician-patient interactions. While there is a sizable amount of data demonstrating that obesity is a state of low-grade inflammation, to our knowledge, there is no single review summarizing its effects on hematologic parameters and thrombotic ris...
journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/ejh.13560
更新日期:2020-12-03 00:00:00
abstract::This report describes a family with TUBB1-associated macrothrombocytopenia diagnosed based on abnormal platelet β1-tubulin distribution. A circumferential marginal microtubule band was undetectable, whereas microtubules were frayed and disorganized in every platelet from the affected individuals. Patients were heteroz...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12252
更新日期:2014-04-01 00:00:00
abstract:BACKGROUND:Most viral infections are known to exert adverse effects on bone marrow function. However, certain viruses have recently been found to be therapeutically beneficial in the treatment of some malignant disorders. METHODS AND MATERIALS:A retrospective study was conducted at the Armed Forces Hospital, Riyadh, S...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2005.00504.x
更新日期:2005-09-01 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:UNLABELLED:Historically, treatment of invasive fungal infections (IFI) has consisted of amphotericin B. However, new therapeutic agents have recently been introduced. At the same time, the relatively low incidence of IFI and the progress in the diagnostic accuracy of IFI have made routine use of empirical antifungal th...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2006.00712.x
更新日期:2006-10-01 00:00:00
abstract::Cytomegalovirus (CMV) infection is one of the most common causes of morbidity and mortality after allogeneic marrow transplantation. We studied 14 consecutive CMV-seropositive patients adding ganciclovir (2.5 mg/kg i.v. every 8 hours for 7 days prior to transplant and 6 mg/kg three times a week after neutrophils becam...
journal_title:European journal of haematology
pub_type: 临床试验,杂志文章
doi:10.1111/j.1600-0609.1991.tb01863.x
更新日期:1991-11-01 00:00:00
abstract::An intensive protocol utilizing mitoxantrone, high-dose cytarabine, vincristine, etoposide and methylprednisolone as induction therapy for chronic myeloid leukemia in blast transformation is described. Fourteen patients were treated, with a remission/second chronic phase achieved in 64%. None of the 3 patients older t...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1996.tb00488.x
更新日期:1996-07-01 00:00:00
abstract::Using a multiparameter flow cytometry assay enumerating cells positive for CD13, CD14 and CD66 antigens, we determined the asynchronous CD14/CD66 co-expression in unselected bone marrow and peripheral blood samples with suspected malignant blood disorders. CD14/CD66 co-expression > or = 5% were found in 131/691 bone m...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1998.tb01098.x
更新日期:1998-11-01 00:00:00
abstract::Cardiac disease is the primary cause of mortality in beta-thalassemia patients. Except for ventricular dysfunction and pulmonary hypertension that represent the main forms of heart disease in these patients, valvular abnormalities including valvular regurgitation, endocardial thickening and calcification and mitral va...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2004.00213.x
更新日期:2004-04-01 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:Disseminated adenovirus (AdV) infections following allogeneic stem cell transplantation (allo-SCT) are increasingly recognised, particularly in children. This study evaluated the clinical relevance of disseminated AdV infections in adult allo-SCT recipients, after different conditioning regimens. METHODS:In...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2007.00821.x
更新日期:2007-04-01 00:00:00
abstract::506 unselected, previously untreated patients with Hodgkin's disease were treated at the Finsen Institute between 1969 and 1983. The prognostic significance of age, sex, stage, systemic symptoms, histologic subtype, number of involved nodal regions, total tumour burden (peripheral + intrathoracic nodal tumour burden, ...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:
更新日期:1989-08-01 00:00:00
abstract:OBJECTIVES:Detection of iron deficiency during pregnancy with hemoglobin (Hb) and serum measurements is insignificant as the measurements may be affected by e.g. hemodilution or accelerated erythropoiesis. This study tests whether cell indices will give a more reliable measure of iron deficiency in pregnant women at te...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2007.00964.x
更新日期:2007-12-01 00:00:00
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 ...
journal_title:European journal of haematology
pub_type: 杂志文章,多中心研究
doi:10.1111/ejh.12885
更新日期:2017-07-01 00:00:00
abstract::We describe a case of acute promyelocytic leukemia in a 61-yr-old woman with a cryptic insertion of RARA gene into PML gene. Using a combination of cytogenetic and molecular methods, we confirmed the insertion and presence of the PML-RARA transcript and lack of the reciprocal RARA-PML transcript. Although such cryptic...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12326
更新日期:2014-10-01 00:00:00
abstract::The methylenetetrahydrofolate reductase (MTHFR) encodes a major enzyme in folate metabolism. It has been suggested that two MTHFR polymorphisms, 677C>T and 1298A>C, influence risk of acute lymphoblastic leukemia (ALL). Most studies on relation of MTHFR polymorphisms to ALL susceptibility have been in pediatric populat...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2010.01430.x
更新日期:2010-06-01 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::Steroid-refractory graft-versus-host disease (GvHD) is a complication following an allogeneic stem cell transplantation with limited therapeutic options. Studies have shown a response in up to 80% of patients with this condition after treatment with the CD25 monoclonal antibody, basiliximab. Despite the good responses...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12691
更新日期:2016-08-01 00:00:00
abstract::To estimate the efficacy of human megakaryocyte purification techniques, mixtures of known numbers of megakaryocytes with a known ploidy range and of bone marrow or peripheral blood mononuclear cells were made. These artificial bone marrow samples were submitted to either a counterflow centrifugal elutriation or Perco...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1991.tb01575.x
更新日期:1991-10-01 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
abstract:AIMS:Platelets retain cytoplasmic messenger RNA and are capable of protein biosynthesis. Several diseases are known to impact the platelet transcriptome but the effect of non-valvular atrial fibrillation (NVAF) on platelet RNA transcript is essentially unknown. The aim of this study was to evaluate the impact of NVAF o...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12879
更新日期:2017-06-01 00:00:00
abstract::To investigate the role of protein phosphorylation in the early phase of EPO-mediated signal transduction, we EPO-stimulated a murine erythroid cell line ELM-I-1 transformed by plasmids comprised of the c-fos enhancer/promoter linked to the luciferase gene. Using this reporter gene system, we previously showed that EP...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1994.tb00647.x
更新日期:1994-04-01 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::MYH9 disorders are autosomal dominant macrothrombocytopenias with leukocyte inclusion bodies. Single point mutations in the protein-coding sequence of the MYH9 gene are the most common cause. So far no large gene deletion/insertion and splicing defects have been reported. Conventional DNA sequencing of each MYH9-codin...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2008.01046.x
更新日期:2008-06-01 00:00:00