Defective spectrin dimer-dimer association in a family with transfusion dependent homozygous hereditary elliptocytosis.

abstract：:We have tested the different mononuclear blood cell populations of seven patients with severe haemophilia and one patient with F VII deficiency for the presence of HBV DNA. These subjects were all polytransfused with non-heated coagulation factors; three were HBsAg positive, five HBsAg negative but anti-HBc and anti-H...

journal_title：British journal of haematology

authors： Lauré F,Chatenoud L,Pasquinelli C,Gazengel C,Beaurain G,Torchet MF,Zagury D,Bach JF,Bréchot C

更新日期：1987-02-01 00:00:00

abstract：:Monoclonal gammopathy of undetermined significance (MGUS) has been associated with an increased risk of thrombosis. We carried out a retrospective multicentre cohort study on 1491 patients with MGUS. In 49 patients (3.3%) MGUS was diagnosed after a thrombotic event. Follow-up details for a period of at least 12 months...

journal_title：British journal of haematology

authors： Za T,De Stefano V,Rossi E,Petrucci MT,Andriani A,Annino L,Cimino G,Caravita T,Pisani F,Ciminello A,Torelli F,Villivà N,Bongarzoni V,Rago A,Betti S,Levi A,Felici S,Gentilini F,Calabrese E,Leone G,Multiple Myeloma G

更新日期：2013-03-01 00:00:00

abstract：:Bone sialoprotein (BSP) is a glycoprotein essentially found in mineralizing connective tissues. We have recently demonstrated that BSP is ectopically expressed by carcinomas that metastasize to bone with high frequency. Multiple myeloma (MM) is characterized by the localization of tumour plasma cells in the bone marro...

journal_title：British journal of haematology

authors： Bellahcène A,Van Riet I,de Greef C,Antoine N,Young MF,Van Camp B,Castronovo V

更新日期：2000-12-01 00:00:00

abstract：:The plasma concentrations of erythropoietin (Ep), soluble transferrin receptors (sTfRs), iron, total iron binding capacity (TIBC) and ferritin were monitored in five leukaemia patients undergoing autologous bone marrow stem cell transplantation (BMSCT) and in 10 lymphoma and 21 ovarian cancer patients undergoing autol...

journal_title：British journal of haematology

authors： Testa U,Rutella S,Martucci R,Scambia G,D'Onofrio G,Pierelli L,Sica S,Benedetti Panici PL,Menichella G,Foti E,Mastroberardino G,Mancuso S,Leone G,Peschle C

更新日期：1997-03-01 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：:The prognostic significance of SOCS3 protein expression was determined in de novo follicular lymphomas (FL) with t(14;18) and bcl-2 overexpression. Presentation lymph nodes from 82 FL patients for whom clinical information was available were immunohistochemically segregated into SOCS3-positive (n = 42) or -negative (n...

journal_title：British journal of haematology

authors： Krishnadasan R,Bifulco C,Kim J,Rodov S,Zieske AW,Vanasse GJ

更新日期：2006-10-01 00:00:00

abstract：:The records of 121 children presenting with acute lymphoblastic leukaemia between 1969 and 1982 were reviewed. The overall relapse free survival rate was 50%. However, girls presenting with a total white blood cell count of 20 X 10(9)/l or less (35% of all patients) had a particularly favourable prognosis with an 80% ...

journal_title：British journal of haematology

authors： Stockley RJ,Ahlquist PY,Mott MG

更新日期：1983-10-01 00:00:00

abstract：:Decreased bone formation plays an important role in the development of lytic lesions during the late stage of multiple myeloma (MM). Release of insulin-like growth factor binding protein-4 (IGFBP4) by tumour cells adjacent to bone may inhibit IGF-I-stimulated osteoblast growth and contribute to decreased bone formatio...

journal_title：British journal of haematology

authors： Feliers D,Woodruff K,Abboud S

更新日期：1999-03-01 00:00:00

abstract：:Risk factors for acute graft-versus-host disease (GvHD) remain controversial. We performed uni- and multivariate statistical analyses on a series of 37 patients receiving a non-depleted allogeneic bone marrow transplant from an HLA-identical sibling donor for a haematological malignancy, in order to identify risk fact...

journal_title：British journal of haematology

authors： Bagot M,Mary JY,Heslan M,Kuentz M,Cordonnier C,Vernant JP,Dubertret L,Levy JP

更新日期：1988-12-01 00:00:00

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

authors： Pan IQ,Hall ER,Wu KK

更新日期：1984-10-01 00:00:00

abstract：:The two major epidemiological clues to the pathogenesis of Burkitt lymphoma (BL) are the geographical association with malaria--BL incidence relates to the malaria transmission rate--and early infection by Epstein-Barr virus (EBV). Both agents cause B cell hyperplasia, which is almost certainly an essential component ...

journal_title：British journal of haematology

authors： Magrath I

更新日期：2012-03-01 00:00:00

abstract：:Proteasome inhibition induces the accumulation of aggregated misfolded/ubiquitinated proteins in the aggresome; conversely, histone deacetylase 6 (HDAC6) inhibition blocks aggresome formation. Although this rationale has been the basis of proteasome inhibitor (PI) and HDAC6 inhibitor combination studies, the role of d...

journal_title：British journal of haematology

authors： Mishima Y,Santo L,Eda H,Cirstea D,Nemani N,Yee AJ,O'Donnell E,Selig MK,Quayle SN,Arastu-Kapur S,Kirk C,Boise LH,Jones SS,Raje N

更新日期：2015-05-01 00:00:00

abstract：:137 patients with severe aplastic anaemia (SAA) were treated in Basel from 1976 to 1986. 34 underwent bone marrow transplantation (BMT) and 103 received antilymphocyte globulin (ALG) therapy. We have analysed the incidence of late haematological complications in both groups of patients. 20 patients treated with ALG de...

journal_title：British journal of haematology

authors： Tichelli A,Gratwohl A,Würsch A,Nissen C,Speck B

更新日期：1988-07-01 00:00:00

abstract：:Two single-nucleotide substitutions in PKLR constituted the molecular basis underlying pyruvate kinase (PK) deficiency in a patient with severe haemolytic anaemia. One novel mutation, IVS5+1G>A, abolished the intron 5 donor splice site. The other mutation, c.1436G>A, altered the intron 10 donor splice site consensus s...

journal_title：British journal of haematology

authors： Wijk R,van Wesel AC,Thomas AA,Rijksen G,van Solinge WW

更新日期：2004-04-01 00:00:00

abstract：:Hydroxyurea (HU), an inhibitor of DNA synthesis, can also induce haemoglobinization in certain erythroid cell lines. In this study, we report that intracellular peroxides levels were increased in HU-treated murine erythroleukaemia (MEL) cells and that l-acetyl-N-cysteine (LNAC), a potent reducing reagent, had a signif...

journal_title：British journal of haematology

authors： Nagai T,Tarumoto T,Miyoshi T,Ohmine K,Muroi K,Komatsu N,Sassa S,Ozawa K

更新日期：2003-05-01 00:00:00

abstract：:Increased osteoclastic bone resorption is the major causal factor of bone disease in multiple myeloma. Recently, non-invasive methods have been developed for the estimation of bone resorptive activity. To evaluate the biological sensitivity and clinical usefulness of five biochemical assays for measuring the C-termina...

journal_title：British journal of haematology

authors： Abildgaard N,Brixen K,Kristensen JE,Eriksen EF,Nielsen JL,Heickendorff L

更新日期：2003-01-01 00:00:00

abstract：:In this study we investigated the proliferation of three well-documented MM lines and 10 bone marrow samples from myeloma patients in response to rh-SCF alone and combined with Interleukin-6 (IL-6), IL-3 and IL-3/GM-CSF fusion protein PIXY 321. Neoplastic plasma cells were highly purified (> 90%) by immunomagnetic dep...

journal_title：British journal of haematology

authors： Lemoli RM,Fortuna A,Grande A,Gamberi B,Bonsi L,Fogli M,Amabile M,Cavo M,Ferrari S,Tura S

更新日期：1994-12-01 00:00:00

abstract：:A 16-year-old boy with a bleeding disorder since infancy has a long bleeding time, normal platelet count and morphology and normal plasma factor-VIII activities. His platelets undergo normal shape change and primary aggregation in response to ADP but show defective 5-hydroxytryptamine (5-HT) secretion and aggregation ...

journal_title：British journal of haematology

authors： Hardisty RM,Machin SJ,Nokes TJ,Rink TJ,Smith SW

更新日期：1983-04-01 00:00:00

abstract：:A total of 229 patients with chronic myeloid leukaemia (CML) in chronic phase were randomized between 1986 and 1990 to receive or not receive additional splenic irradiation as part of their conditioning prior to bone marrow transplantation (BMT). Both groups, 115 patients with and 114 patients without splenic irradiat...

journal_title：British journal of haematology

authors： Gratwohl A,Hermans J,van Biezen A,Arcese W,Debusscher L,Ernst P,Ferrant A,Frassoni F,Gahrton G,Iriondo A,Kolb HJ,Link H,Niederwieser D,Ruutu T,Siegert W,Zwaan FE

更新日期：1996-12-01 00:00:00

abstract：:The pyrimidine analogue cytosine arabinoside (AraC) is one of the most effective drugs used in the treatment of acute leukaemia. Overexpression of the multidrug resistance (MDR-1) gene and its product, P-glycoprotein (P-gp), is associated with cellular resistance to drugs, such as anthracyclines and vinca alkaloids. T...

journal_title：British journal of haematology

authors： Månsson E,Paul A,Löfgren C,Ullberg K,Paul C,Eriksson S,Albertioni F

更新日期：2001-09-01 00:00:00

abstract：:Four children with acute lymphoblastic leukaemia (ALL) who relapsed after allogeneic bone marrow transplantation (BMT) were treated with donor lymphocyte infusion (DLI) without prior conditioning. Three patients had previously received a non-T-cell-depleted matched sibling BMT and the fourth had a T-cell-depleted matc...

journal_title：British journal of haematology

authors： Atra A,Millar B,Shepherd V,Shankar A,Wilson K,Treleaven J,Pritchard-Jones K,Meller ST,Pinkerton CR

更新日期：1997-04-01 00:00:00

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

authors： Deane M,Norton JD

更新日期：1991-03-01 00:00:00

abstract：:Over the past four decades, remarkable progress has been made in the treatment and prognosis of multiple myeloma (MM), although it remains an incurable disease. Chemotherapy resistance is a major hurdle for treatment efficacy. Drug resistance can be innate and so driven by genes involved in the drug metabolism pathway...

journal_title：British journal of haematology

authors： Macauda A,Castelli E,Buda G,Pelosini M,Butrym A,Watek M,Kruszewski M,Vangsted AJ,Rymko M,Jamroziak K,Abildgaard N,Haastrup EK,Mazur G,Ríos R,Jurczyszyn A,Zawirska D,Dudziński M,Raźny M,Dutka M,Tomczak W,Suska A,

更新日期：2018-11-01 00:00:00

abstract：:Routine testing on plasma from a patient due to undergo a coronary artery bypass graft operation revealed a prolonged thrombin clotting time associated with a normal plasma fibrinogen level when this was determined by a method not dependent upon the rate of fibrin formation. Fibrinogen purified from the patient's plas...

journal_title：British journal of haematology

authors： Lane DA,Cuddigan B,VanRoss M,Kakkar VV

更新日期：1980-03-01 00:00:00

abstract：:Adult survivors of childhood acute lymphoblastic leukaemia (ALL) have a four-fold excess risk of mortality from cardiovascular disease. This cardiovascular risk has not been fully characterized. ALL survivors [n = 784, median age 31·7 years (18·9-59·1)] in the St. Jude Lifetime Cohort Study underwent evaluation for ca...

journal_title：British journal of haematology

authors： Nottage KA,Ness KK,Li C,Srivastava D,Robison LL,Hudson MM

更新日期：2014-05-01 00:00:00

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

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

abstract：:Increasing demand for population screening for the haemoglobinopathies gives rise to a requirement for high throughput systems, which allow for cost effective, rapid, sensitive and specific screening of clinically significant haemoglobins. We have developed a practical and efficient approach using tryptic digestion an...

journal_title：British journal of haematology

authors： Daniel YA,Turner C,Haynes RM,Hunt BJ,Dalton RN

更新日期：2005-08-01 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：:Analogous to T cells, Natural Killer (NK) cells may facilitate engraftment, combat infection, and control cancer in bone marrow or haematopoietic stem cell transplantation (HSCT); however, NK cells do not cause graft-versus-host disease. Killer immunoglobulin-like receptors (KIRs) regulate NK cell function, and recent...

journal_title：British journal of haematology

authors： Leung W

更新日期：2011-10-01 00:00:00

abstract：:We have studied the surface expression of the Toll-like receptor family member CD 180 on cells from 78 patients with B-chronic lymphocytic leukaemia (B-CLL). B-CLL cells had variable levels of CD 180 expression, but this was always less than that expressed by normal blood B cells and was stable for 24 months. Signific...

journal_title：British journal of haematology

authors： Porakishvili N,Kulikova N,Jewell AP,Youinou PY,Yong K,Nathwani A,Heelan B,Duke V,Hamblin TJ,Wallace P,Ely P,Clark EA,Lydyard PM

更新日期：2005-11-01 00:00:00