Molecular pathology of haemoglobin H disease in Sardinians.

abstract：:Cytokines and chemokines determine mobilisation of Langerhans cells and their dysregulation is implicated in the pathogenesis of Langerhans cell histiocytosis (LCH). Twenty point mutations of 12 different cytokine genes were studied in 41 Italian children, 15 with single-system (SS) and 26 with multi-system disease. T...

journal_title：British journal of haematology

authors： De Filippi P,Badulli C,Cuccia M,De Silvestri A,Dametto E,Pasi A,Garaventa A,del Prever AB,Todesco A,Trizzino A,Danesino C,Martinetti M,Aricò M

更新日期：2006-03-01 00:00:00

abstract：:The responsiveness of bone marrow erythroid progenitors (CFU-E and BFU-E) to recombinant human erythropoietin (rh-Ep) was investigated in vitro in 21 patients with multiple myeloma to assess the clinical usefulness of rh-Ep in this disease. CFU-E and BFU-E assays were performed by methylcellulose culture methods. The ...

journal_title：British journal of haematology

authors： Aoki I,Nishijima K,Homori M,Nakahara K,Higashi K,Ishikawa K

更新日期：1992-08-01 00:00:00

abstract：:Myeloma cells thrive in an environment of sustained inflammation, which impacts the development and evolution of the disease, as well as drug resistance. We evaluated the impact of genetic polymorphisms in the Toll-like receptor 4 (TLR4) pathway, which have been implicated in different inflammatory responses in the ou...

journal_title：British journal of haematology

authors： Bagratuni T,Terpos E,Eleutherakis-Papaiakovou E,Kalapanida D,Gavriatopoulou M,Migkou M,Liacos CI,Tasidou A,Matsouka C,Mparmparousi D,Dimopoulos MA,Kastritis E

更新日期：2016-01-01 00:00:00

abstract：:During the 3 years 1984-86, 314 cases of multiple myeloma were diagnosed in the Health Care Region of Western Sweden. 180 of these cases were included in a clinical trial; 71 were notified to the trial but excluded; 49 cases were not reported to the trial; 14 were diagnosed post mortem. The crude incidence rate of mye...

journal_title：British journal of haematology

authors： Hjorth M,Holmberg E,Rödjer S,Westin J

更新日期：1992-01-01 00:00:00

abstract：:We investigated the use of a new conditioning regimen followed by allogeneic bone marrow transplantation (BMT) for treating children with acute lymphoblastic leukaemia (ALL) after relapse within 6 months of the completion of therapy. One hundred and sixteen children with acute lymphoblastic leukaemia in second or subs...

journal_title：British journal of haematology

authors： Bordigoni P,Esperou H,Souillet G,Pico J,Michel G,Lacour B,Reiffers J,Sadoun A,Rohrlich P,Jouet JP,Milpied N,Lutz P,Plouvier E,Cornu G,Vannier JP,Gandemer V,Rubie H,Gratecos N,Leverger G,Stephan JL,Boutard P,Vern

更新日期：1998-08-01 00:00:00

abstract：:A highly sensitive Y-microsatellite amplification system was developed for the detection of post bone marrow transplantation (BMT) chimaerism. The system is able to detect the DNA equivalent of a single male cell in a background of 10(5) female cells. For clinical applications it has the distinct advantage that the po...

journal_title：British journal of haematology

authors： Lo YM,Noakes L,Roux E,Jeannet M,Chapuis B,Fleming KA,Wainscoat JS

更新日期：1995-03-01 00:00:00

abstract：:Hodgkin-Reed Sternberg (H-RS) cells harbour clonal immunoglobulin gene (Ig) rearrangements in almost all cases of classical Hodgkin's disease but lack Ig gene expression. In the H-RS cell line L1236, a somatic mutation of the Ig heavy-chain gene promoter octamer motif has been described as a putative reason for absenc...

journal_title：British journal of haematology

authors： Re D,Staratschek-Jox A,Holtick U,Diehl V,Wolf J

更新日期：2001-11-01 00:00:00

abstract：:The replicative potential of human CD56(+) CD3(-) natural killer (NK) cells is unknown. We found that by exposing NK cells to the leukaemic cell line K562 genetically modified to express 4-1BB ligand and interleukin 15 (K562-mb15-41BBL), they expanded for up to 30 population doublings, achieving numbers that ranged fr...

journal_title：British journal of haematology

authors： Fujisaki H,Kakuda H,Imai C,Mullighan CG,Campana D

更新日期：2009-06-01 00:00:00

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

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

abstract：:The levels of soluble CD9 antigen released into spent medium from bone marrow (BM) cells were assayed using a unique enzyme-linked immunosorbent assay. We demonstrated that a considerable amount of soluble CD9 antigen was consistently detected in the spent medium from CD9+ leukaemic blasts, but little from normal or r...

journal_title：British journal of haematology

authors： Komada Y,Zhang SL,Zhou YW,Shibata T,Azuma E,Sakurai M

更新日期：1992-08-01 00:00:00

abstract：:Apoptotic cell death is induced by the cross-linking of Fas/APO-1 receptor (CD95) in acute myelogenous leukaemia (AML) cells. Since CD95 ligand (CD95L) is expressed on interleukin-2 (IL-2)-activated T cells, we investigated the involvement of CD95-CD95L pathway in T cell-mediated cytotoxicity against AML cells. Activa...

journal_title：British journal of haematology

authors： Komada Y,Zhou YW,Zhang XL,Chen TX,Tanaka S,Azuma E,Sakurai M

更新日期：1997-01-01 00:00:00

abstract：:The frequency and prognostic relevance of translocations t(11;14) and t(4;14), the most common translocations involving the immunoglobulin heavy chain (IgH) gene in multiple myeloma (MM), were investigated in 128 patients treated with intensive chemotherapy and autologous stem cell transplant. Myeloma cells were ident...

journal_title：British journal of haematology

authors： Chang H,Sloan S,Li D,Zhuang L,Yi QL,Chen CI,Reece D,Chun K,Keith Stewart A

更新日期：2004-04-01 00:00:00

abstract：:FOXP2 mutation causes a severe inherited speech and language defect, while the related transcription factors FOXP1, FOXP3 and FOXP4 are implicated in cancer. FOXP2 mRNA and protein expression were characterised in normal human tissues, haematological cell lines and multiple myeloma (MM) patients' samples. FOXP2 mRNA a...

journal_title：British journal of haematology

authors： Campbell AJ,Lyne L,Brown PJ,Launchbury RJ,Bignone P,Chi J,Roncador G,Lawrie CH,Gatter KC,Kusec R,Banham AH

更新日期：2010-04-01 00:00:00

abstract：:This study reports the characterization of a spontaneous lymphoblastoid cell line (LCL) raised from the peripheral blood of a patient with Kostmann's congenital neutropenia. The LCL was composed of EBV-infected polyclonal B cells and displayed surface markers and pattern of growth in vitro typical of normal LCLs. The ...

journal_title：British journal of haematology

authors： Corcione A,Roncella S,Cutrona G,Mori PG,Ferrarini M,Pistoia V

更新日期：1993-12-01 00:00:00

abstract：:Globin synthesis was studied in four Negro families including 10 members with Hb A-HPFH and four with Hb S-HPFH. The beta/alpha specific activity ratios in 10 of these HPFH heterozygotes were similar to those of the control group. In two patients with Hb A-HPFH, the beta/alpha ratio was slightly decreased in one (0.84...

journal_title：British journal of haematology

authors： Friedman S,Schwartz E,Ahern E,Ahern V

更新日期：1976-03-01 00:00:00

abstract：:Primary non-engraftment or early rejection after transplantation of haematopoietic stem cells represent life-threatening complications of allogeneic stem cell transplantation. Management of early graft failure has been problematic, as the risk of fatal infectious complications increases with the time of pancytopenia a...

journal_title：British journal of haematology

authors： Schlegel PG,Eyrich M,Bader P,Handgretinger R,Lang P,Niethammer D,Klingebiel T

更新日期：2000-11-01 00:00:00

abstract：:Kidney iron deposition measured by R2* (magnetic resonance imaging) MRI is posited to result from tubular reabsorption of filtered haemoglobin due to intravascular haemolysis. In chronically transfused sickle cell disease (SCD), R2* is elevated and positively correlated with lactate dehydrogenase (LDH). To account for...

journal_title：British journal of haematology

authors： Denton CC,Detterich JA,Coates TD,Wood JC

更新日期：2020-11-20 00:00:00

abstract：:Bone marrow blast cells of 174 child and 188 adult patients with AML were examined and characterized in terms of their FAB type, immunological phenotype (102 children, 123 adults) and karyotype (69 children, 95 adults). The incidence of FAB variants of AML proved similar in children and adults. In patients under 15 an...

journal_title：British journal of haematology

authors： Frenkel MA,Tupitsyn NN,Protasova AK,Kadagidze ZG,Konstantinova LN,Fleischman EW

更新日期：1994-08-01 00:00:00

abstract：:A prospective study was undertaken to determine the effectiveness of an empirical antibiotic therapy regimen in 34 neutropenic patients undergoing bone marrow transplantation or remission-induction chemotherapy for leukaemia. Throughout the study period a total of 90 pyrexial episodes were monitored in which organisms...

journal_title：British journal of haematology

authors： Barnes RA,Rogers TR

更新日期：1987-05-01 00:00:00

abstract：:Two new variants of erythrocytic glucose-6-phosphate dehydrogenase have been found in one German patient and in another patient of Turkish origin. The enzymes were partially purified 165-fold and 111-fold respectively. Both revealed reduced activity, increased thermolability and a pH-optimum in the alkaline region (8....

journal_title：British journal of haematology

authors： Gahr M,Bornhalm D,Schröter W

更新日期：1976-07-01 00:00:00

abstract：:Ernest Beutler was one of the preeminent haematologists of the last half of the 20th and the early 21st century. In a career that spanned six decades, his research interests included such diverse areas as red cell metabolism, blood preservation, glycolipid storage diseases, leukaemias and iron metabolism. Indeed, he w...

journal_title：British journal of haematology

authors： Beutler S,Beutler B

更新日期：2011-03-01 00:00:00

abstract：:Haemophilia B Leyden is characterized by severe factor IX deficiency during childhood with partial resolution at puberty or following the administration of anabolic steroids. The disorder has recently been associated with point mutations in the putative factor IX promoter region, which contains an imperfect direct rep...

journal_title：British journal of haematology

authors： Royle G,Van de Water NS,Berry E,Ockelford PA,Browett PJ

更新日期：1991-02-01 00:00:00

abstract：:Few reports exist concerning heart transplantation in recipients with end-stage myocardiopathy-associated heart failure caused by iron overload occurring with beta-thalassaemia, Diamond-Blackfan syndrome or haemochromatosis. Seven potential transplant candidates (six male, one female, mean age 26 years) with such hear...

journal_title：British journal of haematology

authors： Koerner MM,Tenderich G,Minami K,zu Knyphausen E,Mannebach H,Kleesiek K,Meyer H,Koerfer R

更新日期：1997-05-01 00:00:00

abstract：:In order to investigate whether the clinical behaviour of extranodal natural killer/T-cell lymphoma, nasal type (ENKTL) varies by race within a geographic region, we identified a total of 213 non-Hispanic whites, Hispanics and Asians/Pacific islanders (APIs) diagnosed with ENKTL in the California Cancer Registry betwe...

journal_title：British journal of haematology

authors： Ai WZ,Chang ET,Fish K,Fu K,Weisenburger DD,Keegan TH

更新日期：2012-03-01 00:00:00

abstract：:Translocations involving the MLL gene on the chromosome 11 (11q23) are frequently observed in acute leukaemia. The detection of this genetic change has a unique significance as a result of its implication of poor prognosis. To reveal the utility of fluorescence in situ hybridization (FISH) in detecting the MLL translo...

journal_title：British journal of haematology

authors： Kim HJ,Cho HI,Kim EC,Ko EK,See CJ,Park SY,Lee DS

更新日期：2002-12-01 00:00:00

abstract：:The blood-related cancer leukaemias were the first diseases where human cancer stem cells, or leukaemic stem cells (LSC), were isolated. The haematopoietic system is one of the best tissues for investigating cancer stem cells, because the developmental hierarchy of normal blood formation is well defined. Leukaemias ca...

journal_title：British journal of haematology

authors： Bonnet D

更新日期：2005-08-01 00:00:00

abstract：:Dendritic cells (DC) are attractive candidates for use in vaccine-based immunotherapy. We have analysed the functional capability of DC generated in vitro from blood CD14(+) cells of chronic lymphocytic leukaemia (CLL) patients and healthy donors by culturing for 10 d with granulocyte-macrophage colony-stimulating fac...

journal_title：British journal of haematology

authors： Rezvany MR,Jeddi-Tehrani M,Biberfeld P,Söderlund J,Mellstedt H,Osterborg A,Rabbani H

更新日期：2001-11-01 00:00:00

abstract：:Inherited factor VII (FVII) deficiency is a rare autosomal recessive disorder associated with a bleeding tendency. We describe three patients with congenital FVII deficiency who have been treated with activated recombinant factor VII (rVIIa). Two patients had novel mutations and were treated prophylactically with 1.2 ...

journal_title：British journal of haematology

authors： Mathijssen NC,Masereeuw R,Verbeek K,Lavergne JM,Costa JM,van Heerde WL,Nováková IR

更新日期：2004-05-01 00:00:00

abstract：:Employing an immunoblotting procedure, we have identified and characterized an autoantigen carried on glycoprotein (GP) IIb in a patient with chronic idiopathic thrombocytopenic purpura (ITP), and have compared the location of the autoantigen with that of the platelet-specific alloantigen Baka. Immunoblots, using the ...

journal_title：British journal of haematology

authors： Tomiyama Y,Kurata Y,Shibata Y,Honda S,Furubayashi T,Mizutani H,Tsubakio T,Yonezawa T,Tarui S

更新日期：1989-01-01 00:00:00

abstract：:Little is known about the impact of cytomegalovirus (CMV) infections that occur after human leucocyte antigen (HLA)-matched unrelated donor (MUD) non-myleoablative haematopoietic stem cell transplantation (HCT). We analysed the incidence, onset and outcomes of CMV infections in 59 recipients of MUD and in 109 recipien...

journal_title：British journal of haematology

authors： Junghanss C,Storb R,Maris MB,Carter RA,Sandmaier BM,Maloney DG,McSweeney PA,Corey L,Boeckh M

更新日期：2003-11-01 00:00:00