Abstract:
:Hemoglobin (Hb) Korle-Bu (beta73; Asp-Asn) is the most frequent of the rare beta-chain variants in the population of West Africa whereas Hb E (beta26; Glu-Lys) is common among the Southeast Asian population. We report a hitherto undescribed condition in which these two beta-chain variants co-segregate. The proband was a 19-year-old Thai pregnant woman in her second trimester of pregnancy who visited our thalassemia screening unit. Cellulose acetate electrophoresis and high-performance liquid chromatography (HPLC) analysis of Hb detected one abnormal Hb in addition to the Hb E. Analysis of DNA sequences revealed a GAT-AAT mutation at codon 73 in trans to a GAG-AAG mutation at codon 26 of the beta-globin gene. Polymerase chain reaction (PCR) analysis of the alpha-globin gene cluster of the patient detected a 3.7-kb deletional alpha-thalassemia 2. Family study identified that her mother had the same genotype and her father was a simple Hb E carrier. The hematological data of these unusual cases of hemoglobinopathy are presented and compared with a simple heterozygote for Hb Korle-Bu found in another unrelated Thai family. beta-Globin gene haplotype linked to the Thai beta(Korle-Bu) and a simple DNA assay based on allele-specific PCR for rapid diagnosis of Hb Korle-Bu are also described.
journal_name
Ann Hematoljournal_title
Annals of hematologyauthors
Changtrakun Y,Fucharoen S,Ayukarn K,Siriratmanawong N,Fucharoen G,Sanchaisuriya Kdoi
10.1007/s00277-002-0485-0subject
Has Abstractpub_date
2002-07-01 00:00:00pages
389-93issue
7eissn
0939-5555issn
1432-0584journal_volume
81pub_type
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journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770000209
更新日期:2000-11-01 00:00:00
abstract::Arsenic trioxide (As2O3) is a highly effective agent in the treatment of acute promyelocytic leukemia (APL), whereas other hematopoietic tumors are less responsive to this agent and mechanisms underlying As2O3,-resistance are poorly understood. To better understand the complex network of GSH-related pathways in As2O3 ...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-006-0139-8
更新日期:2006-10-01 00:00:00
abstract::Despite significant advances in the treatment of complications requiring intensive care unit (ICU) admission, ICU mortality remains high for patients after allogeneic stem cell transplantation. We evaluated the role of thrombocytopenia and poor graft function in allogeneic stem cell recipients receiving ICU treatments...
journal_title:Annals of hematology
pub_type: 临床试验,杂志文章
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更新日期:2019-02-01 00:00:00
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journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/BF01715126
更新日期:1992-07-01 00:00:00
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journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-002-0506-z
更新日期:2002-10-01 00:00:00
abstract::A 24-year-old woman with a large cell anaplastic CD 30-positive T-cell non-Hodgkin's lymphoma (NHL) developed downbeat nystagmus, anisocoria, and oscillopsia. Prior to overt cerebral invasion by NHL, she had a thiamine deficiency with very low thiamine concentrations in the CSF, probably caused by protracted vomiting ...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770050484
更新日期:1999-02-01 00:00:00
abstract::This retrospective study evaluated the benefit of alemtuzumab monotherapy in unselected patients with advanced B-cell chronic lymphocytic leukemia (CLL) and prolymphocytic leukemia (B-PLL) to definitely describe the impact of this antibody in clinical routine use. Data were collected from 208 consecutive, mainly pretr...
journal_title:Annals of hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1007/s00277-013-1966-z
更新日期:2014-02-01 00:00:00
abstract::Sickle cell patients are characterized by stress erythropoiesis involving cytokines, growth factors, and adhesion molecules. We set out to determine whether serum soluble vascular cell adhesion molecule-1 (sVCAM-1) levels, which are inversely related to red blood cell counts in sickle cell disease (SCD), reflect eryth...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-003-0610-8
更新日期:2003-03-01 00:00:00
abstract::Despite high-dose chemotherapy and autografting, the outcome for patients with primary refractory Hodgkin's disease (HD) or multiple relapses remains unsatisfactory. Six pediatric patients (median age: 16 years, range: 11-19) received reduced intensity conditioning and allogeneic peripheral blood stem cell transplanta...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-003-0814-y
更新日期:2004-04-01 00:00:00
abstract::Polycythemia vera (PV) is a myeloproliferative neoplasm characterized by overproduction of red blood cells. We have performed a comprehensive characterization of blood immune cells for expression of naïve and memory receptors as well as β2m-associated and β2m-free MHC class I heavy chains, also known as closed and ope...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-018-3332-7
更新日期:2018-09-01 00:00:00
abstract::Reactive oxygen intermediates (ROI) generated in the respiratory burst reaction are crucial for the killing of bacteria and fungi in phagocytes. The key enzyme for the respiratory burst reaction is the NADPH oxidase. Reactive oxygen intermediates have additionally been proposed to be of general importance for the expr...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-003-0837-4
更新日期:2004-04-01 00:00:00
abstract::To characterize T-cell large granular leukemia in Asia, 22 Chinese patients from a single institute were reported, together with an analysis of 88 Asian and 272 Western patients identified from the literature. In our cohort, anemia due to pure red cell aplasia (PRCA) occurred in 15/22 (68%) of cases, being the most co...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-009-0895-3
更新日期:2010-04-01 00:00:00
abstract::Despite recent advances made in its treatment, multiple myeloma (MM) remains an incurable B cell malignancy. Thus, the objective for treating these patients is to prolong overall survival (OS) and preserve patients' quality of life. We have analyzed data from 264 consecutive MM patients who had their initial visit bet...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-016-2888-3
更新日期:2017-03-01 00:00:00
abstract::Established risk factors for thrombosis in essential thrombocythemia (ET) include age (≥ 60 years) and previous vascular events. Recently, also leukocytosis has been proposed in risk stratification of ET patients. We report a retrospective study on 532 ET patients followed for a median of 7.6 years. Sixty-four patient...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-010-1154-3
更新日期:2011-08-01 00:00:00
abstract::Aberrant promoter methylation of RASSF6 and RASSF10 occurs at a high frequency in acute lymphoblastic leukemia (ALL). Because of the complexity of the current minimal residual disease (MRD) detecting-methods, the DNA methylation status of the RASSF6 and RASSF10 genes could potentially become biomarkers for the assessm...
journal_title:Annals of hematology
pub_type: 临床试验,杂志文章
doi:10.1007/s00277-019-03775-y
更新日期:2019-12-01 00:00:00
abstract::Bone marrow transplantation (BMT) and immunosuppression (IS) have improved the prognosis of aplastic anemia; both treatments have specific advantages and drawbacks but similar survival rates. Analysis of additional endpoints may help in treatment decisions. In a single-center study, patients with aplastic anemia treat...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-004-0930-3
更新日期:2005-01-01 00:00:00
abstract::In vivo, growth factors are currently investigated for their capacity to trigger leukemic stem cells into cycle and thus overcome kinetic drug resistance. In this study, the susceptibility of leukemic clonogenic cells to individual growth factors was related to cytosine-arabinoside (Ara-C) sensitivity. The effects of ...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/BF01737421
更新日期:1994-05-01 00:00:00
abstract::Ixazomib, the first oral proteasome inhibitor (PI), has been approved for the treatment of relapsed refractory multiple myeloma (RRMM) in combination with lenalidomide and dexamethasone, based on the TOURMALINE-MM1 phase 3 trial, which demonstrated the efficacy and safety of this all-oral triplet, compared with lenali...
journal_title:Annals of hematology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00277-020-03985-9
更新日期:2020-06-01 00:00:00
abstract::Systemic AL amyloidosis is associated with nearly 15% of cases of multiple myeloma, but data on the frequency and significance of amyloid deposits in the bone marrow of patients affected by multiple myeloma without clinical signs of systemic amyloidosis are scanty. Bone marrow smears of 166 unselected patients affecte...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-009-0857-9
更新日期:2010-05-01 00:00:00
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journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-016-2813-9
更新日期:2016-12-01 00:00:00
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journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-015-2311-5
更新日期:2015-06-01 00:00:00
abstract::Only about one third of all patients with acute myeloid leukemia (AML) will be cured by common chemotherapy regimens. Susceptibility towards chemotherapy either of the leukemic bulk or the leukemic stem cell is considered the major determining parameter for long-term outcome. The purpose of the present study was to in...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-007-0361-z
更新日期:2008-01-01 00:00:00
abstract::We have performed a retrospective analysis of all patients with extragastric mucosa-associated lymphoid tissue (MALT) lymphoma treated at our institution to compare the efficacy of first-line therapeutic modalities including surgery, radiation, systemic therapy, and antibiotics. One hundred eighty-five patients with e...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-014-2042-z
更新日期:2014-08-01 00:00:00
abstract::This study aimed to determine whether dose-dense therapy improves 3-year survival over the standard therapy for untreated aggressive lymphoma. One hundred and fifteen patients with untreated aggressive lymphoma were stratified by center, age, and international prognostic index and randomized to one of two treatment ar...
journal_title:Annals of hematology
pub_type: 杂志文章,随机对照试验
doi:10.1007/s00277-009-0811-x
更新日期:2010-03-01 00:00:00
abstract::We monitored platelet activation by means of P-selectin and platelet monocyte aggregates (PMA) and platelet function by whole blood multiple electrode aggregometry and platelet adhesion under high shear in chronic immune thrombocytopenia patients to define changes in platelet activation during treatment with eltrombop...
journal_title:Annals of hematology
pub_type: 杂志文章,随机对照试验
doi:10.1007/s00277-011-1249-5
更新日期:2012-01-01 00:00:00
abstract::Autologous stem cell transplant (ASCT) is standard consolidation therapy in management of multiple myeloma (MM) patients. We reviewed records of all consecutive MM patients who underwent ASCT with high-dose melphalan at our center from year 2002 to 2016. A total of 141 ASCT were conducted (90 males and 51 females) wit...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-018-3370-1
更新日期:2018-10-01 00:00:00
abstract::Parainfluenza virus (PIV) infection is a significant cause of morbidity and mortality, especially in hematologic malignancy patients including hematopoietic stem cell transplantation (HCT) recipients. However, limited information is available for risk stratification in PIV-infected patients with hematologic malignancy...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-020-03996-6
更新日期:2020-06-01 00:00:00
abstract::Treatment of acute lymphoblastic leukemia (ALL) requires the combination of multiple drugs to integrate a complete remission. The different prognostic factors (age, leukocytes, risk, cytogenetic alterations) allow identifying those patients with a high risk of relapse, but there are few described factors that impact t...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-020-04277-y
更新日期:2020-11-01 00:00:00
abstract::This retrospective single-center study compared thromboembolic and hemorrhagic complications, survival and causes of death in a cohort of 102 consecutive patients with myeloproliferative disorders (MPD). We included 17 patients with essential thrombocythemia (ET), 59 with polycythemia vera (PV), and 26 with osteomyelo...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002779900136
更新日期:2000-06-01 00:00:00
abstract::The presenting features of 356 previously untreated multiple myeloma (MM) patients grouped according to age were analyzed in order (a) to elucidate the possible differences in initial clinical and laboratory features between patients younger than 50 years and the older ones and (b) to statistically assess the prognost...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770050365
更新日期:1998-02-01 00:00:00