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 was cleaved during globin processing but that the N alpha-terminal group was totally acetylated. This resulted in structural modifications of a region crucial for oxygen binding. As a consequence, hemoglobin Lyon-Bron displayed both a reduced chloride effect and a decreased oxygen affinity, this last point explaining the apparent anemia.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Lacan P,Souillet G,Aubry M,Promé D,Richelme-David S,Kister J,Wajcman H,Francina Adoi
10.1002/ajh.10051keywords:
subject
Has Abstractpub_date
2002-03-01 00:00:00pages
214-8issue
3eissn
0361-8609issn
1096-8652pii
10.1002/ajh.10051journal_volume
69pub_type
杂志文章abstract::Regulatory T-cells (Tregs) are increased in chronic lymphocytic leukemia(CLL) and correlates with clinical and biological features of active/progressive disease. However, little is known about their ability to predict the time to first treatment (TFT). We evaluated 75 patients with Rai stage 0 CLL, in whom the absolut...
journal_title:American journal of hematology
pub_type: 信件
doi:10.1002/ajh.23170
更新日期:2012-06-01 00:00:00
abstract::Systemic light-chain (AL) amyloidosis may be associated with acquired factor X (FX) deficiency and optimal management of this coagulopathy is unknown. We reviewed our experience with 60 patients with isolated FX deficiency (< or =50%) due to AL amyloidosis that underwent an invasive procedure between 1975 and 2007. Th...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21603
更新日期:2010-03-01 00:00:00
abstract::It has been suggested that alterations of cell cycle genes probably contribute to the pathogenesis of endemic Burkitt's lymphoma (BL) in addition to c-MYC translocation. Mutations disrupting the normal nuclear localization signal of the retinoblastoma-related gene Rb2/p130 have been documented in BL cell lines and pri...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20749
更新日期:2007-03-01 00:00:00
abstract::Immunoglobulin (Ig) light chain amyloidosis is a clonal but nonproliferative plasma cell disorder in which fragments of an Ig light chain are deposited in tissues. The clinical features depend on the organs involved but can include restrictive cardiomyopathy, nephrotic syndrome, hepatic failure, and peripheral/autonom...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.21934
更新日期:2011-02-01 00:00:00
abstract::Transfusion-related acute lung injury (TRALI) is associated with administration of all plasma containing blood products. We present a 14-year-old adolescent diagnosed with idiopathic thrombocytopenic purpura who developed acute respiratory insufficiency compatible with TRALI within 5 hr following intravenous anti-D. F...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21185
更新日期:2008-08-01 00:00:00
abstract::Chronic transfusion therapy is the treatment of choice for preventing stroke recurrence in children with sickle cell disease (SCD). The majority of children affected by this devastating complication live in the developing world where access to regular blood transfusions may be impractical. Since 2000, in the absence o...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.22142
更新日期:2011-10-01 00:00:00
abstract::This paper describes, for the first time, the utilization of nebulized morphine in the management of severe chest pain in two young adult African-American patients who suffered from generalized acute sickle cell painful episodes. While hospitalized, both patients developed new sharp chest wall pain, and were treated w...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20064
更新日期:2004-06-01 00:00:00
abstract::Protein S (ProS) is a physiological inhibitor of coagulation with an important function in the down-regulation of thrombin generation. ProS deficiency is a major risk factor for venous thrombosis. This study enrolled 40 ProS-deficient probands to investigate the molecular basis of hereditary ProS deficiency in Chinese...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/ajh.23525
更新日期:2013-10-01 00:00:00
abstract::Comparison of the chromosome findings obtained on routine examination (10-50 cells) of the marrows from patients with Ph1-positive CML with those based on a large number (110-500 cells) of metaphases in six of these patients, in whom appropriate material was available, revealed the presence of small percentages of ane...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830030202
更新日期:1977-01-01 00:00:00
abstract::Although treatment with plasma exchange increased the survival of patients with thrombotic thrombocytopenia purpura to 80% in the 1980s, no further increase of survival occurred over the next 20 years. However, more consistent use of adjuvant treatment with corticosteroids and rituximab in recent years has begun to fu...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.23126
更新日期:2012-05-01 00:00:00
abstract::Coagulation factor VIII and von Willebrand factor (VWF) are key proteins in procoagulant activation. Higher FVIII coagulant activity (FVIII :C) and VWF antigen (VWF :Ag) are risk factors for cardiovascular disease and venous thromboembolism. Beyond associations with ABO blood group, genetic determinants of FVIII and V...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/ajh.24005
更新日期:2015-06-01 00:00:00
abstract::A 17-year-old male with bilineal hybrid acute leukemia is described. Two-color flow cytometric analysis of blast surface phenotype revealed that there were two groups of blasts which showed either CD 10+ CD 19+ CD 13- CD 33- or CD 10- CD 19- CD 13+ CD 33+, but not both. He developed a complete remission by treatment w...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830350211
更新日期:1990-10-01 00:00:00
abstract::Splenic lymphoma with villous lymphocytes (SLVL) is a relatively new entity with only a few reports published. We report seven cases of SLVL with detailed clinicopathologic and comprehensive immunophenotypic studies to further characterize this lymphoma, which is frequently confused with hairy cell leukemia and other ...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830450107
更新日期:1994-01-01 00:00:00
abstract::Graft failure is a life-threatening complication after allogeneic hematopoietic stem cell transplantation (HSCT). Graft failure is more prevalent after umbilical cord blood transplantation (UCBT) compared with conventional adult stem cell sources. We identified 21 consecutive patients who experienced graft failure aft...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23845
更新日期:2014-12-01 00:00:00
abstract::Serologic diagnosis for Epstein-Barr virus (EBV) infection is problematic when patients receive exogenous immunoglobulin. We recently diagnosed primary EBV infection by detecting EBV-determined nuclear antigen (EBNA) and EBV-DNA in peripheral blood mononuclear cells (PBMC) using immunofluorescence, in situ hybridizati...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830360416
更新日期:1991-04-01 00:00:00
abstract::Chimeric antigen receptor (CAR) T-cell therapy represents a major advancement in personalized cancer treatment. In this strategy, a patient's own T cells are genetically engineered to express a synthetic receptor that binds a tumor antigen. CAR T cells are then expanded for clinical use and infused back into the patie...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.25418
更新日期:2019-05-01 00:00:00
abstract::Infantile malignant osteopetrosis (IMO) includes various genetic disorders that affect osteoclast development and/or function. Genotype-phenotype correlation studies in IMO have been hampered by the rarity and heterogeneity of the disease and by the severity of the clinical course, which often leads to death early in ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21447
更新日期:2009-08-01 00:00:00
abstract::Binding of chemoattractant to polymorphonuclear leukocytes (PMNL) triggers a series of events like polymerization of actin and tubulin, orientation of cells, chemotaxis, increase in fluid pinocytosis and phagocytosis, and stimulation of microbicidal pathways which includes lysosomal degranulation and generation of rea...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199607)52:3<155::AID-AJH4>
更新日期:1996-07-01 00:00:00
abstract::Eosinophilia may represent an early paraclinical sign of hematological malignant disease, but no reports exist on its predictive value for hematological malignancies. From the Copenhagen Primary Care Differential Count (CopDiff) Database, we identified 356,196 individuals with at least one differential cell count (DIF...
journal_title:American journal of hematology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ajh.23515
更新日期:2013-10-01 00:00:00
abstract::A case of a patient presenting with idiopathic concurrent erythrocytic and megakaryocytic aplasia is reported. The patient's response to immunosuppressive therapy and her bone marrow pathology clearly suggest an immune mechanism. Based on the lack of suppression of erythroid colony growth, several mechanisms are postu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199601)51:1<68::AID-AJH11>
更新日期:1996-01-01 00:00:00
abstract::Little is known about the frequency and significance of clinically unapparent or occult hemorrhage in ITP. Therefore, we prospectively explored the sites and frequency of occult bleeding in children with severe ITP at diagnosis or upon symptomatic relapse in a prospective, single-institution cohort study of patients ≤...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24264
更新日期:2016-03-01 00:00:00
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 ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23414
更新日期:2013-05-01 00:00:00
abstract::Conventional treatment of mantle cell lymphoma (MCL) yields modest responses and short remissions. We report 30 hematopoietic stem cell transplants (HSCT) for MCL: 13 autologous, 10 allogeneic myeloablative, and 7 nonablative. After a median 1.2 years from diagnosis (range 0.5 to 4.7) and a median of 2 pre-HSCT chemot...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20646
更新日期:2006-07-01 00:00:00
abstract::In order to study the influence of oxidized low-density lipoprotein (Ox-LDL) on platelet functional morphology at an early activation stage, washed human blood platelets were stimulated by 100 micrograms/ml Ox-LDL at 37 degrees C. The settling and spreading process of stimulated and unstimulated platelets on Formvar-c...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830490302
更新日期:1995-07-01 00:00:00
abstract::A 56-year-old man was admitted to our hospital with leukocytosis, anemia, and thrombocytopenia. Acute monoblastic leukemia was diagnosed. Two subsequent courses of consolidation chemotherapy consisted of conventional doses of cytarabine and intermediate-dose cytarabine. Intermediate-dose cytarabine was infused intrave...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20772
更新日期:2007-04-01 00:00:00
abstract::Quinine-induced immune thrombocytopenia with hemolytic uremic syndrome (HUS) is a recently defined clinical entity. In this paper we have attempted to characterize the natural history and laboratory abnormalities typical of quinine-induced immune thrombocytopenia associated with hemolytic uremic syndrome in nine patie...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830470407
更新日期:1994-12-01 00:00:00
abstract::Withdrawal: Primary prevention of sickle cell disease using preimplantation genetic testing and in vitro fertilization is cost-effective. The above article, published online on 20 August 2020 in Wiley Online Library (wileyonlinelibrary.com) as an Accepted Article, has been withdrawn by agreement between the authors, t...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.25974
更新日期:2020-08-20 00:00:00
abstract::Allogeneic hematopoietic stem cell transplantation (HSCT) represents the only curative option for primary hemophagocytic lymphohistiocytosis (HLH), a rare disease of infants and young children, characterized by recurrent fever, hepatosplenomegaly, and cytopenia. We report a case of successful engraftment and stable fu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10266
更新日期:2003-02-01 00:00:00
abstract::Bacterial and protozoal infections can cause thrombocytopenia and may mimic idiopathic thrombocytopenic purpura (ITP). Brucella species and Toxoplasma are among the infectious agents with protean clinical manifestations which may induce immune thrombocytopenia. In rare cases, thrombocytopenia can be severe and may res...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10382
更新日期:2003-09-01 00:00:00
abstract::Fifteen recently diagnosed patients with acute leukemias admitted for induction chemotherapy were selected for study. When thrombocytopenic (venous platelet count less than 20 X 10(9)/l) these patients received prophylactic platelet transfusions. A total of 67 platelet transfusion therapies were administered and evalu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830110305
更新日期:1981-11-01 00:00:00