Abstract:
:Severe congenital neutropenia (SCN) is characterized by a maturation arrest of myeloid progenitor cells at the stage of promyelocytes in bone marrow and low levels of mature neutrophils in peripheral blood. To date, little is known regarding the underlying pathomechanism of SCN. A defective response of neutrophil precursors to granulocyte colony-stimulating factor (G-CSF) is a suggested mechanism. In the last few years, we and others described point mutations in the cytoplasmic domain of the G-CSF receptor (G-CSFR) mRNA in a subgroup of SCN patients. In one allele of the G-CSFR gene, a C to T substitution resulted in a change from a glutamine codon to a stop codon. The expected G-CSFR proteins were truncated by 83 to 98 amino acids. In this study, we show that the Lyn and Syk kinases are associated with the G-CSFR in neutrophils from SCN patients with point mutations in the cytoplasmic domain of the G-CSFR mRNA. These findings provide additional proof of the expression of normal G-CSFRs in these patients, because the possible Syk binding motif is located between amino acid 727 and 747 in the G-CSFR.
journal_name
Int J Hematoljournal_title
International journal of hematologyauthors
Kasper B,Tidow N,Welte Ksubject
Has Abstractpub_date
1999-12-01 00:00:00pages
241-7issue
4eissn
0925-5710issn
1865-3774journal_volume
70pub_type
杂志文章abstract::We evaluated endogenous serum erythropoietin (Epo) levels in 14 normal subjects (eight males and six females) after a single 400-ml phlebotomy. The subjects were followed up for 56 days. The hemoglobin (Hb) values of both males and females decreased to a nadir on days 3 to 7 post-phlebotomy. Hb values gradually increa...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:
更新日期:1992-04-01 00:00:00
abstract::Graft-versus-host disease (GvHD) causes severe mucositis, impairs feeding and favors infection. The objective of this study was to identify the impact of GvHD in the oral cavity. We reviewed all consecutive patients who developed oral GvHD after HSCT. The study period was over 14 years. 53 patients were identified. M/...
journal_title:International journal of hematology
pub_type: 杂志文章,meta分析,评审
doi:10.1007/s12185-018-2520-5
更新日期:2018-12-01 00:00:00
abstract::The heart is a rare primary site of lymphoma, and cardiac involvement is thought to bring a poorer prognosis. A framework of known clinical presentations, diagnostic features, disease complications, treatments, and outcomes to improve prognostication was constructed by a systematic review in 2011. However, some aspect...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-020-02881-2
更新日期:2020-07-01 00:00:00
abstract::This study aimed at assessing the relationship between thrombosis, hyperhomocysteinemia and vitamin B12 deficiency using a case-control study carried out in 326 patients with thrombosis (case group) and 351 patients from the same hospital (control group). Apart from the classic risk factors, a number of hematological ...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-011-0825-8
更新日期:2011-04-01 00:00:00
abstract::Thymic mucosa-associated lymphoid tissue (MALT) lymphoma shows distinct immunological characteristics, such as the expression of the IgA isotype, the frequent presence of immunoglobulin abnormalities, and a strong association with autoimmune disease, especially Sjögren's syndrome (SjS). We report a case of thymic MALT...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-009-0324-3
更新日期:2009-06-01 00:00:00
abstract::Recent reports of myelodysplastic syndrome/acute myeloid leukemia (t-MDS/AML) developing after treatment with immunosuppressants and granulocyte colony-stimulating factor (G-CSF) has raised the question of whether previously unrecognized myelodysplastic features had been present or whether actual transformation had oc...
journal_title:International journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1532/ijh97.04178
更新日期:2005-06-01 00:00:00
abstract::APOBEC3G has been identified as an anti-human immunodeficiency virus type 1 (HIV-1) host factor that belongs to the APOBEC superfamily of cytidine deaminases. It deaminates cytidine to uridine in nascent minus-strand viral DNA, inducing G-to-A hypermutation in the plus-strand DNA of HIV-1. The accumulating evidence de...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1532/IJH97.05187
更新日期:2006-04-01 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::Chronic lymphocytic leukemia (CLL) shows a remarkable heterogeneity, with some patients having an almost normal lifespan, others surviving only several months after diagnosis despite intensive therapy. The aim of this study was to investigate the serum thymidine kinase 1 (TK1) concentration in Chinese patients with CL...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-009-0380-8
更新日期:2009-09-01 00:00:00
abstract::Neutropenic enterocolitis is a frequent autopsy finding in adult patients with acute leukemias. The predisposing factors other than neutropenia are not clear. There are also contradictions about treatment. Therefore, this entity still presents a diagnostic and therapeutic dilemma for clinicians. This retrospective stu...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:
更新日期:1997-07-01 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::TAL1/SCL is a prime example of an oncogenic transcription factor that is abnormally expressed in acute leukemia due to the replacement of regulator elements. This gene has also been recognized as an essential regulator of hematopoiesis. TAL1 expression is strictly regulated in a lineage- and stage-specific manner. Suc...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1007/s12185-018-2518-z
更新日期:2019-01-01 00:00:00
abstract::The present study was performed to examine whether the expression of CD64 Fc gamma receptor type I (FcgammaRI) on both neutrophils and monocytes can be modulated by multiple daily administrations of granulocyte colony-stimulating factor (G-CSF) to patients with non-Hodgkin's lymphoma in neutropenia caused by CHOP (cyc...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/BF02983535
更新日期:2004-01-01 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::Shwachman-Diamond syndrome (SDS) is a rare congenital disorder featuring exocrine pancreatic insufficiency, growth retardation, and bone marrow dysfunction. Reports suggest that nearly 25% of all cases are complicated with leukemia. Although stem cell transplantation is the sole option for these patients, successful r...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1532/ijh97.03103
更新日期:2004-02-01 00:00:00
abstract::The Myelodysplastic Syndromes (MDS) represent a group of potentially acute myeloid leukemic disorders. There exists a delicate balance between increased apoptosis and proliferation of the leukemic hematopoietic stem cell that permits many patients to survive for years. When the balance shifts towards proliferation AML...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1007/BF03165122
更新日期:2002-08-01 00:00:00
abstract::Novel approaches to the treatment of haemophilia are needed due to the limitations of the current standard of care, factor replacement therapy. Aspirations include lessening the treatment burden and effectively preventing joint damage. Treating haemophilia by restoring thrombin generation may be an effective approach....
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1007/s12185-018-2548-6
更新日期:2020-01-01 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::We performed a retrospective population-based cohort study of acute myeloid leukemia (AML) in Miyazaki Prefecture, Japan. Over 6 years, we diagnosed 221 patients (211 adults and 10 children) with AML, indicating an incidence of AML in Miyazaki Prefecture of 3.2 per 100,000 per year. In 193 adult patients with non-acut...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-012-1146-2
更新日期:2012-09-01 00:00:00
abstract::The majority of adults diagnosed with acute myeloid leukemia (AML) display acquired cytogenetic aberrations at presentation. Numerous recurring chromosomal abnormalities have been and continue to be identified in AML. In many instances, genes altered by these aberrations have been cloned, providing insights into the m...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:
更新日期:2000-10-01 00:00:00
abstract::Intranuclear proteins, including high mobility group box 1 (HMGB1) and histone H3, released from inflammatory cells activate platelets and the coagulation systems, leading to development of disseminated intravascular coagulation (DIC) in individuals with sepsis. These observations prompted us to hypothesize that HMGB1...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-019-02798-5
更新日期:2020-03-01 00:00:00
abstract::We report 2 paroxysmal nocturnal hemoglobinuria (PNH) patients who were initially diagnosed with aplastic anemia and sequentially developed PNH, myelodysplastic syndromes (MDS), and leukemia. Flow cytometry and cytogenetic analysis showed the initial appearance and expansion of PNH clones, gradual replacement of PNH c...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:
更新日期:2000-08-01 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::Cytomegalovirus (CMV) infection/reactivation is a serious complication after hematopoietic cell transplantation (HCT). The DNA vaccine ASP0113 contains two plasmids encoding CMV antigens (glycoprotein B and tegument phosphoprotein 65) that stimulate humoral and cellular immunity. Between June 2013 and February 2014, A...
journal_title:International journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1007/s12185-016-2110-3
更新日期:2017-02-01 00:00:00
abstract::Immune suppression of hematopoiesis has been implicated in the pathogenesis of acquired aplastic anemia. Similarly, abnormalities of T cells and bone marrow stromal cells have been reported in aplastic anemia, as has abnormal cytokine production. Stem cell factor (SCF) (also known as kit ligand, mast cell growth facto...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:
更新日期:1994-10-01 00:00:00
abstract::Although immunosuppressive therapy using antithymocyte globulin or cyclosporine A (CSA) is effective in selected patients with low-risk myelodysplastic syndrome, the response rates reported so far are inconsistent, and the indication of immunosuppressive therapy for myelodysplastic syndrome has not been clearly define...
journal_title:International journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1532/IJH97.07052
更新日期:2007-08-01 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::Thrombomodulin, encoded by the THBD gene, is a critical regulator of coagulation and innate immunity. Its gene variant (rs3176123, 2729A>C) in the 3' untranslated region has been reported to be associated with vasculopathies. The present study analyzed the impact of THBD variation on transplant outcomes in a cohort of...
journal_title:International journal of hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1007/s12185-015-1852-7
更新日期:2015-10-01 00:00:00
abstract::We describe two patients with primary cardiac malignant lymphoma involving the right atrium and superior vena cava, resulting in intractable right cardiac failure and superior vena cava syndrome. Patients were diagnosed by surgical myocardial biopsy and were treated with combination chemotherapy for non-Hodgkin's lymp...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:
更新日期:1999-10-01 00:00:00
abstract::Acute promyelocytic leukemia (APL) cells express a considerable level of CD33, which is the target of gemtuzumab ozogamicin (GO), and a significantly lower level of P-glycoprotein (P-gp). Therefore, GO is predicted to be a successful treatment for APL. In this article, we report on the GO treatment of 2 patients with ...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1532/IJH97.05069
更新日期:2005-12-01 00:00:00