Abstract:
:Lentiviral modification combined with ex vivo erythroid differentiation was used to stably inhibit RhAG expression, a critical component of the Rh(rhesus) membrane complex defective in the Rh(null) syndrome. The cultured red cells generated recapitulate the major alterations of native Rh(null) cells regarding antigen expression, membrane deformability, and gas transport function, providing the proof of principle for their use as model of Rh(null) syndrome and to investigate Rh complex biogenesis in human primary erythroid cells. Using this model, we were able to reveal for the first time that RhAG extinction alone is sufficient to explain ICAM-4 and CD47 loss observed on native Rh(null) RBCs. Together with the effects of RhAG forced expression in Rh(null) progenitors, this strongly strengthens the hypothesis that RhAG is critical to Rh complex formation. The strategy is also promising for diagnosis purpose in order to overcome the supply from rare blood donors and is applicable to other erythroid defects and rare phenotypes, providing models to dissect membrane biogenesis of multicomplex proteins in erythroid cells, with potential clinical applications in transfusion medicine.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Cambot M,Mazurier C,Canoui-Poitrine F,Hebert N,Picot J,Clay D,Picard V,Ripoche P,Douay L,Dubart-Kupperschmitt A,Cartron JPdoi
10.1002/ajh.23414subject
Has Abstractpub_date
2013-05-01 00:00:00pages
343-9issue
5eissn
0361-8609issn
1096-8652journal_volume
88pub_type
杂志文章abstract::Serum lactic dehydrogenase (LDH) levels are mildly elevated in beta-thalassemia major due to ineffective erythropoiesis. We reviewed the charts of 15 consecutive thalassemic children who underwent allogeneic, T-cell-depleted bone marrow transplantation (BMT) in our department during the last 3 years. Eleven patients h...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199602)51:2<166::AID-AJH13
更新日期:1996-02-01 00:00:00
abstract::Although infectious complications represent a relevant cause of morbidity and mortality in patients with myelofibrosis (MF), little is known about their incidence, outcome and risk factors. We retrospectively evaluated a cohort of 507 MF patients, diagnosed between 1980 and 2014 in five Italian hematology centers, to ...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1002/ajh.24572
更新日期:2017-01-01 00:00:00
abstract::The expression of mature B-cell markers and T markers was determined in lymphocytes isolated from the peripheral blood (PBL) of 20 healthy and 51 patients with non-Hodgkin malignant lymphoma (NHL). The disease was classified as newly diagnosed, in remission, or being treated with chemotherapy and of low-, intermediate...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830260203
更新日期:1987-10-01 00:00:00
abstract::The purpose of the study was to determine if UDP-glucuronosyltransferase (UGT) 2B7 allelic variants encoding for UGT2B7, primary enzyme responsible for morphine glucuronidation contribute to the variability in the hepatic clearance of morphine in sickle cell disease (SCD). Twenty-four hour PK study of morphine and UGT...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21051
更新日期:2008-03-01 00:00:00
abstract::Hemoglobin Lyon-Bron was found in two members of a family of German ascent presenting with a moderate normocytic anemia. In this alpha 2 globin variant, the N-terminal valine of the chain was replaced by an alanine. Electrospray mass spectrometry of the alpha chain showed that, as normally, the initiator methionine wa...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10051
更新日期:2002-03-01 00:00:00
abstract::To elucidate the precise mechanisms of molecular and cellular regulation of hemopoiesis, it is necessary to develop a chemically defined culture assay for purified hemopoietic progenitors. To approach this long-term goal, we attempted to develop a serum-free culture system for enriched human progenitors that permits e...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830280404
更新日期:1988-08-01 00:00:00
abstract::Leukemic cells from the peripheral blood of 52 patients with acute and chronic leukemias were incubated with 12-0-tetradecanoyl phorbol ester (TPA). Thirty-one cases of lymphocytic leukemia (18 cases of acute lymphoblastic and 13 cases of chronic lymphocytic leukemia), 13 cases of acute nonlymphoblastic (myelo or myel...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830130303
更新日期:1982-11-01 00:00:00
abstract::Twenty-eight patients undergoing cardiac surgery were prospectively studied and were assigned to two groups. The patients received 0.8- (Group L) or 2.0-fold (Group H) dose of protamine for the neutralization after cardiopulmonary bypass (CPB) which was determined by Hepcon HMS(R) assay system in which the reagent cha...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/(sici)1096-8652(200006)64:2<112::aid-ajh7>
更新日期:2000-06-01 00:00:00
abstract::Chuvash polycythemia results from a homozygous 598C>T mutation in exon 3 of the von Hippel-Lindau (VHL) gene. This disrupts the normoxia pathway for degrading hypoxia inducible factor (HIF)-1alpha and HIF-2alpha causing altered expression of HIF-1 and HIF-2 inducible genes. As hypoxia induces expression of pro-inflamm...
journal_title:American journal of hematology
pub_type: 杂志文章
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更新日期:2009-02-01 00:00:00
abstract::Levels of many coagulation factors are low in the healthy infant and even lower in the asphyxiated premature infant. We investigated whether a brief exposure to asphyxia at the time of birth causes the activation and consumption of coagulation factors. Following delivery by caesarean section, premature lambs were asph...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830280317
更新日期:1988-07-01 00:00:00
abstract::A family is described in which venous thromboembolic disease is associated with reduced plasma protein C activity and normal levels of protein C antigen. Immunoelectrophoretic analysis of protein C antigen gave an abnormal pattern in all affected members, suggesting that the disorder is related to the presence of a st...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830290410
更新日期:1988-12-01 00:00:00
abstract::A 13-year-old boy presented with progressive bilateral lower limb weakness due to exradural granulocytic sarcoma of the spine. After surgical decompression and radiotherapy, he remained in complete remission 6 years after initial diagnosis. The possible reasons for the favorable outcome of this unique case are discuss...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830220415
更新日期:1986-08-01 00:00:00
abstract::A case of a leukemic transformation following a 27-year history of idiopathic myelofibrosis (IMF) is presented. The patient had two chromosomal abnormalities: a deletion of chromosome 13, del 13(q12q14), and a deletion of chromosome 11, del 11(q14q23). This patient's final diagnosis was acute micromegakaryocytic leuke...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20254
更新日期:2005-01-01 00:00:00
abstract::There is a high prevalence of thalassemia in the Taiwan area. Prenatal diagnosis of severe forms of thalassemia is important for the prevention of this disease. We performed prenatal diagnosis in 167 cases, of which 59 cases were diagnosed by chorionic villi biopsy, 91 cases by amniotic fluid analysis, and 17 cases by...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199706)55:2<65::aid-ajh3>3
更新日期:1997-06-01 00:00:00
abstract::The purpose of the study was to assess consensus and interobserver agreement among an international panel of six hematopathologists regarding characterization and reproducibility of bone marrow (BM) histologic features used to diagnose early stage myeloproliferative neoplasms, in particular differentiation of so-calle...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24837
更新日期:2017-10-01 00:00:00
abstract::Splenectomy is a time-honoured well established approach for patients with steroid-resistant immune thrombocytopenia (ITP). However, due to the more recent availability of therapeutic options alternative to splenectomy, such as rituximab and agonists of the thrombopoietin-receptor, the choice of second-line therapy is...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23823
更新日期:2014-11-01 00:00:00
abstract::Precursor B-ALL blasts may be positive for CD20 in up to 50% of cases. There are few reports on the use of the anti CD20 monoclonal antibody, rituximab, in children with B-ALL. We report on two adult patients with precursor B-ALL who developed significant hepatic toxicity during induction chemotherapy. Single agent ri...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20675
更新日期:2006-12-01 00:00:00
abstract::A patient with chronic myelogenous leukemia (CML) had a Philadelphia chromosome (Ph') [t(9q+;22q-)] in all cells and trisomy C in 13% of cells (later determined to be trisomy 8) at the time of diagnosis. After 21 months of intermittent treatment with busulfan, the acute phase developed with the appearance of an additi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830060208
更新日期:1979-01-01 00:00:00
abstract::Polycythemia vera (PCV) and multiple myeloma are both clonal disorders of hematopoietic stem cells. The simultaneous occurrence of these diseases in an individual patient is rare. A case of synchronous PCV and smoldering myeloma is presented and the literature is reviewed. The issues of clinical importance in this unu...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830440311
更新日期:1993-11-01 00:00:00
abstract::Peptide-pulsed dendritic cells can stimulate T cells showing specific cytotoxicity in chronic myelogenous leukemia. We tried to induce a specific cytotoxic T-cell response stimulated by RNA-pulsed dendritic cells in acute myelogenous leukemia. The total RNA of WEHI-3BD+, a myelomonocytic leukemia cell line derived fro...
journal_title:American journal of hematology
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doi:10.1002/ajh.10471
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abstract::Corticosteroids are essential and one of the mainstays in the treatment of acute lymphoblastic leukemia (ALL). In vitro assays show that dexamethasone(DXM) is five to six times more cytotoxic to leukemic lymphoblasts than prednisolone (PDN) [1], and the use of DXM as an alternative drug for PDN is an important issue i...
journal_title:American journal of hematology
pub_type: 信件,随机对照试验
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更新日期:2010-10-01 00:00:00
abstract::Severe pruritus is frequently associated with myeloproliferative and other systemic illnesses, and often fails to respond to conventional measures. We used danazol (Danocrine), a synthetic attenuated androgen, in the treatment of severe pruritus refractory to conventional therapy. Eight patients had myeloproliferative...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/(SICI)1096-8652(199602)51:2<112::AID-AJH3>
更新日期:1996-02-01 00:00:00
abstract::Neonatal alloimmune thrombocytopenia is an uncommon but important cause of thrombocytopenia in infants. Because of the severity of the thrombocytopenia, some of these infants will have intracranial hemorrhage with resultant long-term disability. Obstetricians and neonatologists have recommended delivery by caesarean s...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830280207
更新日期:1988-06-01 00:00:00
abstract::We describe a newly detected alpha-thalassemia-2 (alpha-thal-2) deletion characterized by a small -2.7-kb deletion involving the alpha 1 globin gene. This deletion has thus far been observed in only one Chinese subject with Hb H disease. ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830380321
更新日期:1991-11-01 00:00:00
abstract::We studied the prognostic value of parameters of angiogenesis on bone marrow biopsies in newly diagnosed multiple myeloma (MM) patients. Angiogenesis parameters studied were the microvessel count done manually on light microscopy (MVD-A), microvessel count done by using computerized image analyzer (MVD-B), and total v...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20639
更新日期:2006-09-01 00:00:00
abstract::We have developed a murine monoclonal antibody (mAb) specific for the delta chain of hemoglobin (Hb) A2 that does not cross-react with alpha, beta, or gamma chains. The mAb reacted with Hb P-Nilotic (beta delta hybrid), but not with Hb Lepore-Boston (delta beta hybrid), indicating an epitope consisting of positions 11...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830380311
更新日期:1991-11-01 00:00:00
abstract::Imatinib mesylate radically changed the natural history of chronic myeloid leukemia (CML). The recent availability of alternative tyrosine kinase inhibitors (TKIs) renders the clinical management of CML more complex. In this article, we summarize our long-term single institution experience. From 2003 to 2012, 102 pati...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1002/ajh.23804
更新日期:2014-10-01 00:00:00
abstract::Endothelin-1 (Et-1) is a vasoconstrictor produced by endothelial and vascular smooth muscle cells in response to hypoxia, which induces hypertrophy in cultured cardiac myocytes. We measured plasma Et-1 levels and left ventricular dimensions in 13 patients with sickle cell anemia (SCD) and in 12 African-American contro...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199807)58:3<195::aid-ajh6>
更新日期:1998-07-01 00:00:00
abstract::Most reports of prognosis and therapy in diffuse small non-cleaved cell lymphoma (DSNCL), an aggressive high-grade non-Hodgkin's lymphoma (NHL) which appears to be of two histopathologic subtypes, have included predominantly a pediatric population and very few adults. We studied 20 newly diagnosed, previously untreate...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830460408
更新日期:1994-08-01 00:00:00
abstract::In patients with light-chain myeloma or primary AL-amyloidosis, 24-hr light-chain excretion in the urine is considered an essential marker of the tumor mass. However, 24-hr urine collection and analysis may be cumbersome and prone to inaccuracy. Recently, a sensitive immunonephelometric assay for immunoglobulin free l...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20007
更新日期:2004-04-01 00:00:00