Residual expression of functional MHC class II molecules in twin brothers with MHC class II deficiency is cell type specific.

Abstract:

:We examined major histocompatibility complex (MHC) class II expression in B cells, peripheral blood monocytes, activated T cells, epidermal Langerhans cells, monocyte-derived dendritic cells, dermal microvascular endothelial cells (DMEC) and fibroblasts of twin brothers with MHC class II deficiency. Although residual human leucocyte antigen (HLA)-DR expression was found on a subpopulation of epidermal Langerhans cells and a subset of peripheral blood monocyte-derived dendritic cells, the patients' B cells, monocytes and activated T cells were HLA-DR negative. After treatment with interferon-gamma (IFN-gamma), the patients' DMEC expressed HLA-DR but not -DP and -DQ at the protein and mRNA level, whereas IFN-gamma failed to induce HLA-DR expression on dermal fibroblasts. The patients' monocyte-derived dendritic cells were capable of processing and presenting tetanus toxoid to autologous T cells, and patient-derived DMEC induced the proliferation of allogeneic CD4(+) T cells in an MHC class II-restricted fashion, indicating that the observed residual MHC class II surface expression was functional. The findings reported show that the defect encountered in these patients is not necessarily expressed to the same extent in different cell lineages, which is relevant for the understanding of the patients' phenotype and also illustrates that only small amounts of MHC class II are needed to mount a functional cellular immune response in vivo.

journal_name

Br J Haematol

authors

Wolf HM,Thon V,Gulle H,Lechleitner S,Eibl MM,Petzelbauer P

doi

10.1046/j.1365-2141.2001.03105.x

subject

Has Abstract

pub_date

2001-11-01 00:00:00

pages

460-71

issue

2

eissn

0007-1048

issn

1365-2141

pii

3105

journal_volume

115

pub_type

杂志文章
  • Multilineage involvement in hypereosinophilic syndrome terminating in granulocytic sarcoma and leukaemic transformation with trisomy 8.

    abstract::We report a patient with hypereosinophilic syndrome (HES), which, 8 years later, transformed into granulocytic sarcoma in the brain and, subsequently, into acute myelocytic leukaemia. Repeated chromosome analyses showed a normal karyotype, until the time of leukaemic transformation when trisomy 8 was confirmed in cell...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.2002.03922.x

    authors: Fujii N,Ikeda K,Takahashi N,Kojima K,Kobayashi Y,Ashiba A,Takenaka K,Fukuda S,Shinagawa K,Ishimaru F,Niiya K,Miura I,Tanimoto M,Harada M

    更新日期:2002-12-01 00:00:00

  • The effect of cyclosporine on haematological parameters in patients with paroxysmal nocturnal haemoglobinuria.

    abstract::Four patients with paroxysmal nocturnal haemoglobinuria (PNH) were treated with cyclosporine. The treatment with cyclosporine was based on the hypothesis that immune-mediated bone-marrow damage is the common pathogenetic mechanism of aplasia and PNH, with lack of GPI-linked ligands for an immune attack (i.e. LFA-3, CD...

    journal_title:British journal of haematology

    pub_type: 临床试验,杂志文章

    doi:10.1111/j.1365-2141.1995.tb08907.x

    authors: van Kamp H,van Imhoff GW,de Wolf JT,Smit JW,Halie MR,Vellenga E

    更新日期:1995-01-01 00:00:00

  • Primary thrombocythaemia associated with systemic mastocytosis: a report of five cases.

    abstract::Mastocytosis is an uncommon disease characterized by a proliferation of tissue mast cells involving various organs, particularly the bone marrow. Though rare, the association of mastocytosis and haematological malignancies is well established. However, the frequency of mastocytosis reported in patients with essential ...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1991.tb08011.x

    authors: Le Tourneau A,Gaulard P,D'Agay MF,Vainchencker W,Cadiou M,Devidas A,Haioun C,Clauvel JP,Audouin J,Diebold J

    更新日期:1991-09-01 00:00:00

  • Autologous bone marrow transplantation with 4-hydroperoxycyclophosphamide purging for acute myeloid leukaemia beyond first remission: a 10-year experience.

    abstract::Between January 1987 and January 1997, 69 eligible patients with acute myeloid leukaemia (AML) in either second (CR2) or third (CR3) complete remission (CR2 = 60, CR3 = 9) underwent 4-hydroperoxycyclophosphamide-purged autologous bone marrow transplantation (BMT) at the Johns Hopkins Oncology Center. The patients' med...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.2002.03530.x

    authors: Smith BD,Jones RJ,Lee SM,Piantadosi S,Vala MS,Fuller D,Gore SD,Noga SJ,O'Donnell PV,Braine H,Vogelsang GB,Fuchs EJ,Flinn IW,Brodsky RA,Ambinder RF,Miller CB

    更新日期:2002-06-01 00:00:00

  • Three unrelated Gaucher's disease patients with three novel point mutations in the glucocerebrosidase gene (P266R, D315H and A318D).

    abstract::Three novel point mutations were detected in the glucocerebrosidase gene of three unrelated Gaucher's disease patients by direct sequencing of PCR products. The first is a C to G change at position 4263 in the genomic sequence (exon 7) which results in a proline to arginine change at position 266 in the mature enzyme ...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1995.tb05298.x

    authors: Walley AJ,Ellis I,Harris A

    更新日期:1995-10-01 00:00:00

  • Alteration of platelet responses to metabolites of arachidonic acid by oral contraceptives.

    abstract::The effects of oral contraceptive (OC) usage on platelet responsiveness to arachidonic acid metabolites was investigated. Platelets obtained from women who had been taking oral contraceptives for at least 3 months were compared with those of age-matched controls. Both the basal and prostacyclin (PGI2)-stimulated plate...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1984.tb06090.x

    authors: Pan IQ,Hall ER,Wu KK

    更新日期:1984-10-01 00:00:00

  • Immunoglobulin gene 'fingerprinting': an approach to analysis of B lymphoid clonality in lymphoproliferative disorders.

    abstract::Rearrangement of the immunoglobulin heavy chain (IgH) gene is widely exploited as a marker of B cell lineage and clonality in the pathology of lymphoproliferative disorders. We have developed a simple, polymerase chain reaction (PCR) based method for detecting IgH gene rearrangement which relies on the observation tha...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1991.tb08570.x

    authors: Deane M,Norton JD

    更新日期:1991-03-01 00:00:00

  • Prospects for primary stroke prevention in children with sickle cell anaemia.

    abstract::This review will focus on the strengths and limitations associated with the current standard of care for primary prevention of ischaemic strokes in children with sickle cell anaemia (SCA) - transcranial Doppler ultrasound (TCD) screening followed by regular blood transfusion therapy when TCD measurement is above a thr...

    journal_title:British journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1111/j.1365-2141.2011.09005.x

    authors: Jordan LC,Casella JF,DeBaun MR

    更新日期:2012-04-01 00:00:00

  • A new type of hereditary persistence of fetal haemoglobin (HPFH): HPFH Tunisia beta + (+C-200)G gamma.

    abstract::Nd-HPFH are haematological conditions which are natural models to aid understanding of the haemoglobin (Hb) switch. In this paper we describe a new non-deletional hereditary persistence of fetal haemoglobin (nd-HPFH) associated with the highest Hb F level observed to date (up to 49% without haemopoietic stress). Seque...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:

    authors: Pissard S,M'rad A,Beuzard Y,Roméo PH

    更新日期:1996-10-01 00:00:00

  • A basophilic micro-organism infecting human red cells.

    abstract::Clusters and chains of basophilic micro-organisms were found on the red cells of a woman who suffered from malignant melanoma. The infection was clinically silent for at least 7 years. During her last year of life the patient became febrile and anaemic. Her spleen enlarged and the basophilic bodies on her red cells be...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1975.tb01824.x

    authors: Clark KG

    更新日期:1975-02-01 00:00:00

  • A molecular study of the t(4;14) in multiple myeloma.

    abstract::The t(4;14) translocation is found in approximately 10% of myeloma patients and results in the deregulation of at least two genes, MMSET and fibroblast growth factor receptor 3 (FGFR3), with the formation of a fusion product between MMSET and the immunoglobulin heavy chain (IgH) locus and overexpression of FGFR3. We h...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.2002.03618.x

    authors: Sibley K,Fenton JA,Dring AM,Ashcroft AJ,Rawstron AC,Morgan GJ

    更新日期:2002-08-01 00:00:00

  • Ber-H2 (anti-CD30)-saporin immunotoxin: a new tool for the treatment of Hodgkin's disease and CD30+ lymphoma: in vitro evaluation.

    abstract::An immunotoxin containing an anti-CD30 monoclonal antibody (Ber-H2) and saporin, a ribosome-inactivating protein type 1, is described. It specifically inhibits protein synthesis by Hodgkin derived target cell lines with a very high efficiency (IC50 ranging from 5 x 10(-12) M to 5 x 10(-14) M, as saporin), while irrele...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1992.tb08208.x

    authors: Tazzari PL,Bolognesi A,de Totero D,Falini B,Lemoli RM,Soria MR,Pileri S,Gobbi M,Stein H,Flenghi L

    更新日期:1992-06-01 00:00:00

  • Major histocompatibility complex abnormalities in non-Hodgkin lymphomas.

    abstract::An optimal antitumoral immune response requires the participation of both CD8 and CD4 T lymphocytes, which are activated by peptide antigen presentation via human leucocyte antigen (HLA) class I and class II molecules respectively. Loss of HLA molecules has been observed in different malignancies, and provides a mecha...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.2002.03814.x

    authors: Drénou B,Le Friec G,Bernard M,Pangault C,Grosset JM,Lamy T,Fauchet R,Amiot L

    更新日期:2002-11-01 00:00:00

  • Stage I/II follicular lymphoma: spread of bcl-2/IgH+ cells in blood and bone marrow from primary site of disease and possibility of clearance after involved field radiotherapy.

    abstract::Stage I/IIA follicular lymphoma (FL) is considered a localised disease that can be adequately treated with radiotherapy alone. Bone marrow (BM) and peripheral blood (PB) involvement in FL was investigated by polymerase chain reaction (PCR) in a series of 24 consecutive patients with histologically revised diagnosis an...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.2007.06545.x

    authors: Pulsoni A,Starza ID,Frattarelli N,Ghia E,Carlotti E,Cavalieri E,Matturro A,Tempera S,Rambaldi A,Foà R

    更新日期:2007-05-01 00:00:00

  • An improved micro-method for obtaining chromosome preparations from individual haemopoietic colonies.

    abstract::A robust method for obtaining chromosome preparations from individual haemopoietic colonies in semi-solid media is described. The accumulation of metaphases and the hypotonic treatment of cells were carried out in the culture dish and individual colonies were transferred onto poly-L-lysine-treated slides and fixed ste...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.1999.01428.x

    authors: Kadhim MA,Pocock DA,Lorimore SA,Wright EG

    更新日期:1999-06-01 00:00:00

  • Influence of clinical status on the efficiency of stored platelet transfusion.

    abstract::The efficiency of stored platelet transfusion was evaluated in terms of clinical status in 141 thrombocytopenic patients. In a paired prospective study in which fresh platelets were used as controls, clinical efficiency was assessed on the basis of the ability to increase platelet count (recovery) and the time to the ...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1994.tb03262.x

    authors: Norol F,Kuentz M,Cordonnier C,Beaujean F,Haioun C,Vernant JP,Duédari N

    更新日期:1994-01-01 00:00:00

  • The blood group I and i antigens of amniotic fluid. I. Association of I and i antigens with blood group A, B and H antigens.

    abstract::Human amniotic fluid has been shown to contain blood group i as well as I antigens. Crude extracts of amniotic fluids at 16-23 weeks of gestation were in general more active than those obtained at term. A pool of amniotic fluids which had A, B, H as well as I and i activity was fractionated with an insolubilized anti-...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1975.tb01863.x

    authors: Feizi T,Cederqvist LL,Childs R

    更新日期:1975-08-01 00:00:00

  • Pulmonary function in thalassaemia major and its correlation with body iron stores.

    abstract::This study compared pulmonary function tests (PFTs) with cardiac, pancreatic and liver iron in 76 thalassemia major (TM) patients. Restrictive lung disease was observed in 16%, hyperinflation in 32% and abnormal diffusing capacity in 3%. While no patients met Global Initiative for Chronic Lung Disease criteria for air...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.2011.08808.x

    authors: Sohn EY,Noetzli LJ,Gera A,Kato R,Coates TD,Harmatz P,Keens TG,Wood JC

    更新日期:2011-10-01 00:00:00

  • Plasma D-dimer levels and their relationship to serum fibrinogen/fibrin degradation products in hypercoagulable states.

    abstract::Plasma D-dimer was measured and compared with serum fibrinogen/fibrin degradation product levels (FDPs) in patients with disseminated intravascular coagulation (DIC) and other conditions associated with a hypercoagulable state. D-dimer (N less than 200 ng/ml) was elevated in all 43 patients with DIC, in 48 of 59 patie...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1989.tb06276.x

    authors: Wilde JT,Kitchen S,Kinsey S,Greaves M,Preston FE

    更新日期:1989-01-01 00:00:00

  • Hereditary non-spherocytic haemolytic anaemia due to red blood cell glutathione synthetase deficiency in four unrelated patients from Spain: clinical and molecular studies.

    abstract::In four unrelated patients with chronic haemolysis and markedly reduced red blood cell (RBC) glutathione (49.5%, 12.6%, 11.5% and 15% of the normal concentration respectively), a severe glutathione synthetase (GSH-S, EC 6.3.2.3) deficiency was found. One case exhibited a neonatal haemolytic anaemia associated with oxo...

    journal_title:British journal of haematology

    pub_type: 杂志文章,评审

    doi:10.1046/j.1365-2141.2001.02526.x

    authors: Corrons JL,Alvarez R,Pujades A,Zarza R,Oliva E,Lasheras G,Callis M,Ribes A,Gelbart T,Beutler E

    更新日期:2001-02-01 00:00:00

  • Analysis of natural killer-associated antigens in peripheral blood and bone marrow of multiple myeloma patients and prognostic implications.

    abstract::The aim of this study was to analyse the expression of NK-associated antigens in both peripheral blood and bone marrow lymphocytes from a large series of newly diagnosed multiple myeloma patients. 112 patients with untreated multiple myeloma (MM) were included in the study. 36 sex- and age-matched healthy volunteers w...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.1996.4651006.x

    authors: García-Sanz R,González M,Orfão A,Moro MJ,Hernández JM,Borrego D,Carnero M,Casanova F,Bárez A,Jiménez R,Portero JA,San Miguel JF

    更新日期:1996-04-01 00:00:00

  • Characterization of murine monoclonal antibodies directed against the Kell blood group glycoprotein.

    abstract::Murine monoclonal antibodies (MoAbs) were produced against the blood group KEL1 glycoprotein (93 kD component) immunopurified from human erythrocytes. One monoclonal antibody, 5A11 (IgGa, kappa), detects by immunoblotting a 93 and 184 kD component from KEL: 1,-2 or KEL: -1,2 red cell membrane preparations, separated b...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1991.tb04539.x

    authors: Jaber A,Loirat MJ,Willem C,Bloy C,Cartron JP,Blanchard D

    更新日期:1991-10-01 00:00:00

  • A single-centre prospective study of clinical and haemostatic risk factors for venous thromboembolism following lower limb arthroplasty.

    abstract::Previous studies report conflicting results concerning the potential significance of thrombophilic genotypes in postarthroplasty venous thromboembolism (VTE). This study assessed thrombophilic genotypes, haemostatic and clinical variables as independent risk factors for VTE postarthroplasty. A total number of 569 pati...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.2005.05419.x

    authors: Joseph JE,Low J,Courtenay B,Neil MJ,McGrath M,Ma D

    更新日期:2005-04-01 00:00:00

  • A new preconditioning regimen with melphalan, busulphan and total body irradiation followed by low-dose immunosuppressant in allogeneic haemopoietic stem cell transplantation.

    abstract::Twenty adult patients with high-risk leukaemia underwent allogeneic haemopoietic stem cell transplantation after melphalan, busulphan and total body irradiation followed by short-term methotrexate and low-dose cyclosporine or tacrolimus. Three patients developed veno-occlusive disease and no patient developed renal dy...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.1999.01424.x

    authors: Murata M,Nishida T,Haneda M,Kanie T,Taji H,Iida H,Suzuki R,Hamaguchi M,Minami S,Kodera Y

    更新日期:1999-06-01 00:00:00

  • Genomic organisation and regulation of murine alpha haemoglobin stabilising protein by erythroid Kruppel-like factor.

    abstract::Alpha haemoglobin stabilising protein (AHSP) binds free alpha-globin chains and plays an important role in the protection of red cells, particularly during beta-thalassaemia. Murine ASHP was discovered as a GATA-1 target gene and human AHSP is directly regulated by GATA-1. More recently, AHSP was rediscovered as a hig...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.2006.06381.x

    authors: Keys JR,Tallack MR,Hodge DJ,Cridland SO,David R,Perkins AC

    更新日期:2007-01-01 00:00:00

  • Aminophospholipid exposure, microvesiculation and abnormal protein tyrosine phosphorylation in the platelets of a patient with Scott syndrome: a study using physiologic agonists and local anaesthetics.

    abstract::The Scott syndrome is a rare inherited haemorrhagic disorder characterized by the inability of blood cells to expose aminophospholipids and to shed microparticles. We have had the opportunity to study a recently reported French patient with this syndrome and have confirmed by means of a fluorescence assay for transbil...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2141.1997.5003302.x

    authors: Dachary-Prigent J,Pasquet JM,Fressinaud E,Toti F,Freyssinet JM,Nurden AT

    更新日期:1997-12-01 00:00:00

  • Analysis of 2013 European LeukaemiaNet (ELN) responses in chronic phase CML across four frontline TKI modalities and impact on clinical outcomes.

    abstract::This study assessed the relevance of 2013 European LeukaemiaNet (ELN) response categories on patients treated with common frontline tyrosine kinase inhibitors (TKI) in chronic myeloid leukaemia in chronic phase (CML-CP). Four hundred and eighty-seven patients treated with imatinib (400 mg; IM 400, n = 70; 800 mg; IM80...

    journal_title:British journal of haematology

    pub_type: 临床试验,杂志文章,多中心研究

    doi:10.1111/bjh.13936

    authors: Jain P,Kantarjian H,Sasaki K,Jabbour E,Dasarathula J,Nogueras Gonzalez G,Verstovsek S,Borthakur G,Wierda W,Kadia T,Dellasala S,Pierce S,Ravandi F,O'Brien S,Cortes J

    更新日期:2016-04-01 00:00:00

  • An assessment of an amidolytic assay for factor VII in the laboratory control of oral anticoagulants.

    abstract::A comparison has been made between the prothrombin time test using British Comparative Thromboplastin (BCT) and a chromogenic substrate assay for factor VII in the assessment of laboratory control of oral anticoagulants in short-term and long-term patients. Opportunity was also taken to compare the findings with paral...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2141.1981.tb07198.x

    authors: Poller L,Thomson JM,Bodzenta A,Easton AC,Latallo ZS,Chmielewska J

    更新日期:1981-09-01 00:00:00

  • Increased leucocyte apoptosis in transfused β-thalassaemia patients.

    abstract::This exploratory study assessed apoptosis in peripheral blood leucocytes (PBL) from β-thalassaemia patients receiving chronic transfusions and chelation therapy (deferasirox or deferoxamine) at baseline, 1, 6, and 12 months. At baseline, thalassaemic PBLs presented 50% greater levels of Bax (BAX), 75% higher caspase-3...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/bjh.12076

    authors: Walter PB,Porter J,Evans P,Kwiatkowski JL,Neufeld EJ,Coates T,Giardina PJ,Grady RW,Vichinsky E,Olivieri N,Trachtenberg F,Alberti D,Fung E,Ames B,Higa A,Harmatz P,Thalassemia Clinical Research Network.

    更新日期:2013-02-01 00:00:00

  • Impact of centralized diagnostic review on quality of initial staging in Hodgkin lymphoma: experience of the German Hodgkin Study Group.

    abstract::Accurate clinical staging is crucial for adequate risk-adapted treatment in Hodgkin lymphoma (HL) to prevent patients from under- or over-treatment. Within the latest German Hodgkin Study Group trial generation, diagnostic findings such as histopathology, computerized tomography imaging and clinical risk factors were ...

    journal_title:British journal of haematology

    pub_type: 杂志文章

    doi:10.1111/bjh.13646

    authors: Bröckelmann PJ,Goergen H,Fuchs M,Kriz J,Semrau R,Baues C,Kobe C,Behringer K,Eichenauer DA,von Tresckow B,Klimm B,Halbsguth T,Wongso D,Plütschow A,Haverkamp H,Dietlein M,Eich HT,Stein H,Diehl V,Borchmann P,Engert A

    更新日期:2015-11-01 00:00:00