Abstract:
:Hemoglobin E-beta thalassemia (Hb E/β-thalassemia) is a distinct, yet common, type of β-thalassemia, in which the patient co-inherits a β-thalassemia allele from one parent, and a structural variant, Hb E, from the other parent. This co-inheritance leads to remarkable clinical heterogeneity, varying degrees of chronic anemia, and a wide spectrum of complications due to ineffective erythropoiesis and iron overload. Hydroxyurea (HU), an oral chemotherapeutic drug, is expected to decrease disease severity. To assess the clinical efficacy and safety of HU in Hb E/β-thalassemia patients. We searched MEDLINE, EMBASE, Cochrane databases, and major preceding conferences for studies that assessed HU in Hb E/β-thalassemias patients. The effect size was estimated as a proportion (responder/sample size). Qualities of eligible studies were assessed using NIH tools. A total of five [one randomized clinical trial (RCT) and four observational] studies involving 106 patients were included. HU was associated with a significant RR of 46% with no statistical heterogeneity. No serious adverse effects were reported. Patients with Hb E/β-thalassemia may benefit from a trial of HU, though large RCTs assessing efficacy should be conducted to confirm the findings of this meta-analysis and to assess long-term toxicity and response sustainability.
journal_name
Int J Hematoljournal_title
International journal of hematologyauthors
Algiraigri AH,Kassam Adoi
10.1007/s12185-017-2307-0subject
Has Abstractpub_date
2017-12-01 00:00:00pages
748-756issue
6eissn
0925-5710issn
1865-3774pii
10.1007/s12185-017-2307-0journal_volume
106pub_type
杂志文章,meta分析,评审abstract::According to the 2008 WHO classification, the category of myelodysplastic/myeloproliferative neoplasms (MDS/MPN) includes atypical chronic myeloid leukaemia (aCML), chronic myelomonocytic leukaemia (CMML), MDS/MPN-unclassifiable (MDS/MPN-U), juvenile myelomonocytic leukaemia (JMML) and a "provisional" entity, refracto...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1007/s12185-014-1670-3
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abstract::Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare subtype of myeloid neoplasm. Clonal evolution in the development of BPDCN remains to be elucidated. In the present study, we examined clonal evolution in a case of BPDCN by analyzing the distribution of gene mutations in tumor cells and non-tumor blood cel...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-018-2461-z
更新日期:2018-10-01 00:00:00
abstract::Allogeneic hematopoietic stem cell transplantation (alloSCT) is currently the only curative treatment modality for myelodysplastic syndromes (MDS). The treatment paradigm for MDS has changed in recent years with the introduction of hypomethylating agents (HMAs). The present retrospective multicenter study was designed...
journal_title:International journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1007/s12185-014-1549-3
更新日期:2014-01-01 00:00:00
abstract::A 37-year-old male from Kagoshima Prefecture was admitted with adult T-cell leukemia (ATL). Monoclonal integration of HTLV-1 proviral DNA was found, but the integration site was different from that of terminal deoxynucleotidyltransferase (TdT)-positive MT-1 cells (an ATL cell line). The ATL cells expressed enzymatical...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:
更新日期:1991-02-01 00:00:00
abstract::Early thrombocytopenia is a common hematological abnormality in sick neonates. Here, we examined the relationship between early thrombocytopenia in neonates and parameters associated with thrombopoiesis to identify predictive factors at birth. Two hundred and forty-four neonates admitted to the neonatal intensive care...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-009-0482-3
更新日期:2010-03-01 00:00:00
abstract::Initial treatment with androgen (metenolone acetate) alone for 19 weeks had no effect in a 45-year-old Japanese female with refractory anemia (RA). The patient achieved trilineage hematologic recovery after addition of recombinant human granulocyte colony-stimulating factor (G-CSF) and recombinant human erythropoietin...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1016/s0925-5710(96)00530-0
更新日期:1996-12-01 00:00:00
abstract::We describe a 6-year-old girl and her mother with dominant beta-thalassemia due to hemoglobin Hradec Kralove (Hb HK). Both patients presented microcytic anemia, jaundice, splenomegaly, cholelithiasis, and recurrent hemolytic bouts. Osmotic resistance tests using saline and coiled planet centrifugation revealed the inc...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/BF02983557
更新日期:2003-11-01 00:00:00
abstract::Myeloproliferative neoplasms (MPNs) constitute a group of phenotypically diverse chronic myeloid malignancies, characterized by clonal hematopoiesis and excessive production of terminally differentiated myeloid blood cells. The MPNs include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibro...
journal_title:International journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1007/s12185-013-1295-y
更新日期:2013-03-01 00:00:00
abstract::CD5+ diffuse large B-cell lymphoma (DLBCL) has recently been identified as a subgroup with different clinical characteristics from CD5- DLBCL and as having a poorer outcome than CD5- DLBCL. Data regarding differences in gene alteration between CD5+ and CD5- DLBCL have accumulated. In this article, we report an analysi...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1532/ijh97.04119
更新日期:2005-01-01 00:00:00
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journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1016/s0925-5710(96)00531-2
更新日期:1996-12-01 00:00:00
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journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1532/ijh97.04017
更新日期:2004-10-01 00:00:00
abstract::MicroRNAs (miRNAs) are non-coding regulatory RNAs consisting of 20-24 nucleotides. Over 4,500 miRNAs have been identified in humans, and it is known that nearly all human protein-encoding genes can be controlled by miRNAs in both healthy and malignant cells. Abnormal miRNA expression is known to occur in many cancers,...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1007/s12185-014-1535-9
更新日期:2014-01-01 00:00:00
abstract::The prognostic significance of Bcl-2, Bcl-6, p53, topoisomerase II, and β-tubulin expression was evaluated in diffuse large B-cell lymphoma (DLBCL) patients treated with cyclophosphamide, doxorubicin, vincristine, prednisolone, and rituximab. Eight-year progression-free survival (PFS, P = 0.006) and overall survival (...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-015-1911-0
更新日期:2016-02-01 00:00:00
abstract::Mountain sickness (MS) occurs among humans visiting or inhabiting high altitude environments. We conducted genetic analyses of the AKT3, ANGPTL4, eNOS3 and VEGFA genes in lowland (Han) and highland (Tibetan) Chinese. Ten single nucleotide polymorphisms (SNPs) were evaluated in Han and Tibetan patients with acute (A) a...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-012-1117-7
更新日期:2012-08-01 00:00:00
abstract::To determine useful prognostic factors in treating childhood acute lymphoblastic leukemia (ALL), we correlated conventional risk factors and bone marrow response 14 days after induction chemotherapy. Our study included 116 precursor B-cell (n = 104) and T-cell (n = 12) ALL patients treated with our protocol between 19...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1532/ijh97.04114
更新日期:2005-04-01 00:00:00
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journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-015-1882-1
更新日期:2016-01-01 00:00:00
abstract::Classical Burkitt lymphoma/leukemia (BL/L) presenting L3 morphology is found in 1% of childhood ALL. Recently, it has been described that secondary abnormalities could influence the prognosis of these patients. However, little information is available on these cytogenetic abnormalities and their prognostic importance ...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-010-0757-8
更新日期:2011-02-01 00:00:00
abstract::A patient with chronic myelogenous leukemia (CML) in chronic phase (CP) had been treated with a syngeneic bone marrow transplantation (BMT). Cytogenetic remission was confirmed 3 months later. One year after transplantation, hematological remission persisted while cytogenetic analysis revealed a recurrence of Philadel...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1016/0925-5710(96)00486-0
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abstract::Cold agglutinin disease (CAD) is a rare autoimmune hemolytic anemia, classified into primary and secondary types. Secondary CAD accompanies infection or malignant disease, most often lymphoma, whereas primary CAD frequently represents a lymphoproliferative bone marrow disorder characterized by clonal expansion of B ce...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-012-1158-y
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journal_title:International journal of hematology
pub_type: 临床试验,杂志文章
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abstract::The Wilms tumor gene WT1 is expressed in leukemias and various kinds of solid tumors, including lung and breast cancer, and exerts an oncogenic function in these malignancies, suggesting that WT1 protein is a novel, overexpressed tumor antigen. The WT1 protein, in fact, is an attractive tumor rejection antigen in anim...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1007/BF02982574
更新日期:2002-08-01 00:00:00
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journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:
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journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1016/0925-5710(96)00457-4
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journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-008-0209-x
更新日期:2008-12-01 00:00:00
abstract::Both stem cells and progenitor cells are present in umbilical cord blood (UCB) at a high frequency, making these cells a major target population for experimental and clinical studies. As the use of autologous or allogeneic hematopoietic stem cell transplantation in the treatment of various diseases has grown rapidly i...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1007/s12185-010-0619-4
更新日期:2010-07-01 00:00:00
abstract::Inorganic polyphosphate (polyP) is abundant in biological organisms. PolyP is a major component of dense granules of human platelets and is secreted upon platelet activation. Studies from our lab and others have shown that polyP is a potent modulator of the blood clotting cascade, acting as a pro-hemostatic, prothromb...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1007/s12185-012-1054-5
更新日期:2012-04-01 00:00:00
abstract::A 29-year-old woman presented to the emergency department with exhaustion, fatigue, and abdominal pain. She reported having received a diagnosis of bulimia nervosa 10 years before. On examination, she had a marked pallor and was severely malnourished. Laboratory analysis revealed a dramatically low hemoglobin level of...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1532/IJH97.E0505
更新日期:2005-11-01 00:00:00
abstract::The detection of HIV-1 proviral DNA and genomic RNA was performed by polymerase chain reaction (PCR) in hemophiliacs treated with non-heated clotting factor concentrates. Reamplification with double PCR was performed on those samples that were negative for single PCR. Primer pairs of the gag, env, and pol regions were...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:
更新日期:1993-10-01 00:00:00
abstract::We recently developed an Invader assay combined with reverse transcriptase polymerase-chain-reaction in order to quantify T315I bcr-abl transcripts. Using this assay, we serially monitored T315I bcr-abl transcripts in chronic myeloid leukemia (CML) patients whose bcr-abl transcripts were still detectable at 6 months a...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-009-0290-9
更新日期:2009-05-01 00:00:00
abstract::The prognosis of pulmonary toxoplasmosis, including disseminated toxoplasmosis involving the lungs, following hematopoietic stem cell transplantation (HSCT) is extremely poor due to the difficulties associated with early diagnosis and the rapidly progressive deterioration of multiorgan function. In our institution, we...
journal_title:International journal of hematology
pub_type: 杂志文章
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更新日期:2016-12-01 00:00:00