Abstract:
:Integrins, a family of noncovalently associated alpha beta heterodimeric adhesion receptors, are involved in a variety of pathological and physiological processes. The importance of integrins is evident in the severe pathogenic consequences of their congenital deficiencies: Glanzmann thrombasthenia (GT) and leukocyte adhesion deficiency. In this review, I have focused on GT, a rare autosomal recessive bleeding disorder characterized by the quantitative and/or qualitative abnormality of integrin alpha IIb beta 3 (glycoprotein IIb-IIIa). Molecular genetic analysis of GT, when caused by a quantitative abnormality of alpha IIb beta 3, provides important information regarding key structures for alpha IIb beta 3 biosynthesis. Of particular interest is GT when caused by a qualitative abnormality of alpha IIb beta 3 (GT variants). The analyses of GT variants provide new insight into the regulation of alpha IIb beta 3 function and the interaction between alpha IIb beta 3 and its ligands. This research could contribute to new and better alpha IIb beta 3 antagonists with minimal complications (such as bleeding and thrombocytopenia) for the prevention and treatment of pathological thrombosis.
journal_name
Int J Hematoljournal_title
International journal of hematologyauthors
Tomiyama Ysubject
Has Abstractpub_date
2000-12-01 00:00:00pages
448-54issue
4eissn
0925-5710issn
1865-3774journal_volume
72pub_type
杂志文章,评审abstract::Persistent infection with human parvovirus B19 (B19) is primarily associated with chronic bone marrow failure in immunocompromised patients, but occasionally this organism may also affect immunocompetent hosts. B19 is also suggested as a causative agent of organ failure during bone marrow transplantation (BMT). We her...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1532/ijh97.03161
更新日期:2004-05-01 00:00:00
abstract::A new complicated Ph1 translocation involving five chromosomes, t(9;22;21;11;inv ins(12)-(q15p12p13))(q34;q11;q22;q13;q15), was found in a 64-year-old Korean woman with chronic myelocytic leukemia (CML). At presentation, the patient was found to be in the accelerated phase, she entered the chronic phase after six cycl...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:
更新日期:1991-12-01 00:00:00
abstract::Hypocalcemic cardiomyopathy in primary or secondary hypoparathyroidism is usually refractory to conventional treatment of cardiac failure. We report the case of a thalassemic patient with severe cardiac failure that might have been attributed to several factors, such as hemosiderosis, hypomagnesemia, and hypocalcemia,...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1532/IJH97.E0532
更新日期:2006-05-01 00:00:00
abstract::Hepcidin, the iron regulatory hormone, has three isoforms; -20, -22 and -25. While hepcidin-25 has been studied extensively, the physiological significance of other isoforms remains poorly understood. Using a quantitative method based on liquid chromatography-tandem mass spectrometry (LC-tandem MS) developed by our gr...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-015-1885-y
更新日期:2016-01-01 00:00:00
abstract::A series of expression microarray analyses of chronic myeloid leukemia (CML) by means of complementary DNA microarray representing a set of 1024 human genes were initiated to evaluate the role of expression microarray analysis in CML and to identify CML-associated genes. Among the target genes, 388 differentially expr...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/BF02982130
更新日期:2002-05-01 00:00:00
abstract::The causes of thrombosis in antiphospholipid syndrome (APS) remain unknown, though several hypotheses in regard to hypofibrinolysis have been proposed. To clarify the mechanism, we measured plasma levels of thrombin-activatable fibrinolysis inhibitor (TAFI) in APS patients. Both the TAFI antigen (TAFI:Ag) level measur...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-010-0590-0
更新日期:2010-06-01 00:00:00
abstract::Intrathecal administration of methotrexate (IT-MTX) can lead to neurotoxicity. MTX-induced neurotoxicity occasionally manifests with a stroke-like presentation that is difficult to distinguish from genuine stroke. We retrospectively reviewed records of nine patients with leukemia or lymphoma and episodes of stroke-lik...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-018-2525-0
更新日期:2018-12-01 00:00:00
abstract::Allogeneic stem cell transplantation is an established treatment modality for a variety of hematologic malignancies. Unfortunately it carries a high risk of complications and toxicities related to the intensive preparative regimen which is traditionally used for pre-transplant myeloablation and the graft versus host d...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1007/BF03165241
更新日期:2002-08-01 00:00:00
abstract::Kinetic resistance is assumed to be one of the main mechanisms of drug resistance in acute myeloid leukemia (AML), but the relationship between cell cycle status at diagnosis and achievement of complete remission (CR) is controversial. Based on the possibility that the cell cycle data after starting induction chemothe...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:
更新日期:2000-04-01 00:00:00
abstract::Hemoglobin (Hb) E is the commonest beta-globin variant in Thailand. Other uncommon variants have been rarely reported. We performed direct DNA sequencing of the entire beta-globin gene in cases showing unidentified bands by isolectric focusing electrophoresis or high-performance liquid chromatography. Six different be...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-009-0334-1
更新日期:2009-06-01 00:00:00
abstract::Primary effusion lymphoma (PEL) is a rare type of extranodal lymphoma, typically of a B-cell origin, which presents as lymphomatous effusion with no nodal enlargement or tumor masses. The development PEL is universally associated with human herpes virus-8 (HHV-8) infection. Cases of HHV-8-negative primary lymphomatous...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-018-2426-2
更新日期:2018-09-01 00:00:00
abstract::Aplastic anemia (AA) and pure red cell aplasia (PRCA) appear to be more prevalent in Asian countries including Korea. However, there are no exact data regarding its prevalence and frequency of allogeneic hematopoietic cell transplantation (HCT) in Korea. Here, we present demographic data relating to AA/PRCA/MDS in Kor...
journal_title:International journal of hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1007/s12185-017-2280-7
更新日期:2017-10-01 00:00:00
abstract::Transfusion is believed to be the main cause of iron overload in Japan. A nationwide survey on post-transfusional iron overload subsequently led to the establishment of guidelines for iron chelation therapy in this country. To date, however, detailed clinical information on the entire iron overload population in Japan...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-016-2141-9
更新日期:2017-03-01 00:00:00
abstract::Thirty patients with hematologic diseases received allogeneic hematopoietic stem cell transplants (HSCT) from related donors other than genotypically HLA-matched siblings. Their outcomes were compared with those of 102 patients who had received HSCT from genotypically HLA-matched siblings. All donors in the study grou...
journal_title:International journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1007/BF02982696
更新日期:2002-11-01 00:00:00
abstract::Bronchiolitis obliterans (BO) after allogeneic stem cell transplantation (allo-SCT) is a late-onset, life-threatening respiratory complication that significantly reduces a patient's quality of life. We retrospectively analysed the incidence of and risk factors for BO in allo-SCT recipients. In 2087 patients who underw...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-011-0809-8
更新日期:2011-03-01 00:00:00
abstract::In addition to reduced-intensity conditioning, which has expanded the eligibility for hematopoietic cell transplantation (HCT) to older patients, increased availability of alternative donors, including HLA-mismatched unrelated donors, has increased access to allogeneic HCT for more patients. However, acute graft-versu...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1007/s12185-013-1421-x
更新日期:2013-09-01 00:00:00
abstract::Umbilical cord blood (UCB) is well known to be a rich source of hematopoietic stem cells with practical and ethical advantages. Because mesenchymal stem cells (MSCs) from bone marrow have been regarded as good materials for cell/gene therapy as well as for tissue engineering because of their multidifferentiation poten...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1532/ijh97.a10404
更新日期:2005-02-01 00:00:00
abstract::Aberrant methylation in promoter-associated CpG islands has been recognized as a major mechanism for tumor suppressor gene silencing in several malignancies. We determined the methylation status of nine tumor suppressor genes in 68 newly diagnosed MM patients by methylation-specific PCR. The frequency of promoter hype...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-009-0459-2
更新日期:2010-01-01 00:00:00
abstract::There is no standard treatment for adolescents aged 15 years or older with acute lymphoblastic leukemia (ALL), although this age group has been reported as having a poorer prognosis compared to younger patients. We retrospectively analyzed the outcomes of three consecutive Tokyo Children's Cancer Study Group ALL trial...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-014-1622-y
更新日期:2014-08-01 00:00:00
abstract::Peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) comprises a group of heterogeneous lymphomas that do not fit any other identified PTCL-subgroup and show poor prognosis. To clarify clinical aspects of Japanese PTCL-NOS patients, the Hokkaido Hematology Study Group conducted a multicenter retrospective ana...
journal_title:International journal of hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1007/s12185-013-1383-z
更新日期:2013-08-01 00:00:00
abstract::Posttransplant lymphoproliferative disorder (PTLD) after allogeneic hematopoietic cell transplantation (HCT) is usually donor derived, associated with Epstein-Barr virus (EBV), and of B-cell origin. T-cell PTLD (T-PTLD) after allogeneic HCT is extremely rare. Four of 1015 (0.39%) allogeneic HCT patients were diagnosed...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-020-02890-1
更新日期:2020-08-01 00:00:00
abstract::Autophagy (also known as macroautophagy) is a lysosomal degradation pathway for the clearance of cellular materials, which manifests as an adaptive response to stress stimuli. Over the past decade, numerous studies have linked autophagy with cancer initiation, progression, and chemoresistance. Autophagy defects in nor...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1007/s12185-018-2414-6
更新日期:2018-05-01 00:00:00
abstract::The study was aimed to explore the efficacy and safety of allo-HSCT with high-dose cyclophosphamide-induced immune tolerance for SAA. In the present study, 20 cases (12 male, 8 female; average age = 17.8 years) received reduced-intensity conditioning allo-HSCT from August 2012 to August 2014 in the Beijing Military Re...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-016-2106-z
更新日期:2016-12-01 00:00:00
abstract::We identified two afibrinogenemic girls in two Japanese families and performed molecular analysis to clarify the mechanisms of fibrinogen defects. Genetic analyses were performed by PCR amplification of the fibrinogen gene and DNA sequence analysis. To analyze the mechanisms of mature fibrinogen defects in plasma, we ...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-012-1100-3
更新日期:2012-07-01 00:00:00
abstract::IKZF1 encodes a transcription factor involved in B-cell maturation and differentiation. We genotyped 218 diffuse large B-cell lymphoma (DLBCL) patients and 715 unrelated controls using a TaqMan allelic discrimination assay. No statistical difference was observed in the genotype distribution of the IKZF1 rs4132601 poly...
journal_title:International journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1007/s12185-017-2315-0
更新日期:2017-12-01 00:00:00
abstract::Embryonic stem (ES) cells are derived from blastocysts and are pluripotent. This pluripotency has attracted the interest of numerous researchers, both to expand our fundamental understanding of developmental biology and also because of potential applications in regenerative medicine. Systems biological studies have de...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1007/s12185-010-0517-9
更新日期:2010-04-01 00:00:00
abstract::Post-transplant lymphoproliferative disorders (PTLDs) are serious, life-threatening complications of solid-organ transplantation (SOT) and bone marrow transplantation, and are associated with high mortality. PTLDs represent a heterogeneous group of lymphoproliferative diseases, which show a spectrum of clinical, morph...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-012-1244-1
更新日期:2013-01-01 00:00:00
abstract::A 68-year-old female was diagnosed with acute myeloid leukemia (AML-M2 without 8/21 translocation) in December 2006. Although a complete remission (CR) was obtained after induction chemotherapy, the first post-remission therapy was discontinued because of severe cardiovascular complications. She had a relapse of AML w...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-013-1481-y
更新日期:2014-02-01 00:00:00
abstract::Bortezomib is a proteasome inhibitor demonstrating substantial activity in multiple myeloma. One of its key toxicities is peripheral neuropathy, which is reversible in most patients. The possibility that bortezomib might in rare cases induce severe neuropathies by auto-inflammatory mechanisms remains controversial. We...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-011-0847-2
更新日期:2011-06-01 00:00:00
abstract::We retrospectively investigated increases in large granular lymphocytes (LGL) in peripheral blood during dasatinib treatment in 25 chronic myelogenous leukemia patients. Fifteen of 25 patients (60 %) showed an increase in LGL. All 15 of these patients also showed an increase in NK cells, and 11 showed an increase in C...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-012-1132-8
更新日期:2012-09-01 00:00:00