A novel deletion of the L-ferritin iron-responsive element responsible for severe hereditary hyperferritinaemia-cataract syndrome.

abstract：:Interleukin-6 (IL6) plays a central role in multiple myeloma pathogenesis and confers resistance to corticosteroid-induced apoptosis. We therefore evaluated the efficacy and safety of siltuximab, an anti-IL6 monoclonal antibody, alone and in combination with dexamethasone, for patients with relapsed or refractory mult...

journal_title：British journal of haematology

authors： Voorhees PM,Manges RF,Sonneveld P,Jagannath S,Somlo G,Krishnan A,Lentzsch S,Frank RC,Zweegman S,Wijermans PW,Orlowski RZ,Kranenburg B,Hall B,Casneuf T,Qin X,van de Velde H,Xie H,Thomas SK

更新日期：2013-05-01 00:00:00

abstract：:Well-established histopathological prognostic factors are lacking in primary central nervous system (CNS) lymphomas (PCNSL). The present study investigated the presence and prognostic role of tumour necrosis (TN) and reactive perivascular T-cell infiltrate (RPVI), defined as a rim of small reactive T-lymphocytes occur...

journal_title：British journal of haematology

authors： Ponzoni M,Berger F,Chassagne-Clement C,Tinguely M,Jouvet A,Ferreri AJ,Dell'Oro S,Terreni MR,Doglioni C,Weis J,Cerati M,Milani M,Iuzzolino P,Motta T,Carbone A,Pedrinis E,Sanchez J,Blay JY,Reni M,Conconi A,Bertoni F

更新日期：2007-08-01 00:00:00

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

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

更新日期：2007-01-01 00:00:00

abstract：:Chemokines are a family of over 40 small (8 kDa) related proteins with the function of moving cells along a chemotactic gradient, either to organise cells within an organ or to facilitate the movement of leucocytes around the body. Mouse models have implicated the importance of the chemokine CXCL12 in haematopoiesis a...

journal_title：British journal of haematology

authors： Laurence AD

更新日期：2006-02-01 00:00:00

abstract：:Studies of ethnic disparities in malignancies have revealed variation in clinical outcomes. In multiple myeloma (MM), previous literature has focused only on patients of Caucasian and African-American (AA) descent. We present a Surveillance Epidemiology and End Results (SEER)-based outcome analysis of MM patients from...

journal_title：British journal of haematology

authors： Ailawadhi S,Aldoss IT,Yang D,Razavi P,Cozen W,Sher T,Chanan-Khan A

更新日期：2012-07-01 00:00:00

abstract：:This study aimed to assess the potential of human cord blood (CB) cells to engraft in the xenogenic non-obese diabetic/severe combined immunodeficient (NOD/SCID) mouse model after in vitro expansion culture. We also studied the quality of human haemopoiesis arising from the transplantation of fresh or expanded cells i...

journal_title：British journal of haematology

authors： Denning-Kendall PA,Evely R,Singha S,Chapman M,Bradley BA,Hows JM

更新日期：2002-01-01 00:00:00

abstract：:We developed a novel method for the chemical modification of cytokines with synthetic polymers to increase the therapeutic efficacy of the former in vivo. A pH-reversible amino-protective reagent, dimethylmaleic anhydride (DMMAn), was used for modification of interleukin-6 (IL-6) with polyethylene glycol (PEG). The no...

journal_title：British journal of haematology

authors： Tsunoda S,Ishikawa T,Watanabe M,Kamada H,Yamamoto Y,Tsutsumi Y,Hirano T,Mayumi T

更新日期：2001-01-01 00:00:00

abstract：:Phosphatidylserine (PS) externalization may contribute to Sickle Cell Disease (SCD) characteristics including thrombogenesis, endothelial adhesion and shortened red blood cell (RBC) lifespan. Aminophospholipid translocase (APLT) returns externalized PS to the inner membrane, and phospholipid scramblase (PLSCR) equilib...

journal_title：British journal of haematology

authors： Barber LA,Palascak MB,Joiner CH,Franco RS

更新日期：2009-08-01 00:00:00

abstract：:Anaplastic large cell lymphomas (ALCLs) are rare CD30+ peripheral T-cell lymphomas (PTCLs) classified according to the expression of the anaplastic lymphoma kinase (ALK+) protein or not (ALK-). We have analysed the outcome and risk factors for survival in a population-based bi-national cohort of patients with systemic...

journal_title：British journal of haematology

authors： Cederleuf H,Bjerregård Pedersen M,Jerkeman M,Relander T,d'Amore F,Ellin F

更新日期：2017-09-01 00:00:00

abstract：:This study systematically reviewed and meta-analysed the prognostic value of complete remission status at end-of-treatment (18) F-fluoro-2-deoxy-d-glucose positron emission tomography (FDG-PET) in diffuse large B-cell lymphoma (DLBCL) patients treated with rituximab, cyclophosphamide, doxorubicin, vincristine and pred...

journal_title：British journal of haematology

authors： Adams HJ,Nievelstein RA,Kwee TC

更新日期：2015-07-01 00:00:00

abstract：:Unfractionated heparin (UFH) and low-molecular weight heparin (LMWH) are well defined anticoagulant agents. Recent data suggest that both LMWH and UFH may also have potent anti-inflammatory properties; however, their mechanism of action responsible for the anti-inflammatory effect is not yet fully elucidated. This stu...

journal_title：British journal of haematology

authors： Hochart H,Jenkins PV,Smith OP,White B

更新日期：2006-04-01 00:00:00

abstract：:Purpura fulminans (PF) is a cutaneous manifestation of a dramatic and deadly syndrome of systemic disseminated intravascular coagulation (DIC). It is characterized by microvascular thrombosis in the dermis followed by perivascular haemorrhage. Since two other related syndromes involve the protein C (PC) system, we und...

journal_title：British journal of haematology

authors： Madden RM,Gill JC,Marlar RA

更新日期：1990-05-01 00:00:00

abstract：:Platelet function was evaluated in eight patients with chronic granulocytic leukaemia (CGL), seven Ph1 positive and one Ph1 negative. Seven of the eight patients' platelets had an absence of the second wave of adrenaline induced aggregation on at least one occasion, while five had impaired collagen aggregation. The pl...

journal_title：British journal of haematology

authors： Gerrard JM,Stoddard SF,Shapiro RS,Coccia PF,Ramsay NK,Nesbit ME,Rao GH,Krivit W,White JG

更新日期：1978-12-01 00:00:00

abstract：:Graft-versus-host disease (GVHD) is a major cause of transplant-related mortality (TRM) after allogeneic haematopoietic stem cell transplantation (HSCT) and presents a challenge in haploidentical HSCT. GVHD may be prevented by ex vivo graft T-cell depletion or in vivo depletion of proliferating lymphocytes. However, b...

journal_title：British journal of haematology

authors： Roy DC,Lachance S,Cohen S,Delisle JS,Kiss T,Sauvageau G,Busque L,Ahmad I,Bernard L,Bambace N,Boumédine RS,Guertin MC,Rezvani K,Mielke S,Perreault C,Roy J

更新日期：2019-09-01 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：:Patients with polycythaemia vera and secondary polycythaemia studied with 125I-labelled fibrinogen, had a significantly increased plasma fibrinogen turnover. The abnormalities were greater in polycythaemia vera and correlated with platelet count but not with packed cell volume. In six patients with polycythaemia vera,...

journal_title：British journal of haematology

authors： Boughton BJ,Dallinger KJ

更新日期：1983-01-01 00:00:00

abstract：:The role of autologous stem cell transplantation (ASCT) and the optimal timing of such transplants in patients with follicular lymphoma (FL) remains contentious. We present a single-centre experience documenting the outcomes of 70 FL patients who underwent BEAM (carmustine, cytarabine, etopside, melphalan)-conditioned...

journal_title：British journal of haematology

authors： Kothari J,Peggs KS,Bird A,Thomson KJ,Morris E,Virchis AE,Lambert J,Goldstone AH,Linch DC,Ardeshna KM

更新日期：2014-05-01 00:00:00

abstract：:Thirty-two patients with meningeal leukaemia who achieved meningeal remission with intrathecal methotrexate (MTX) plus dexamethasone (DMT) were entered in a randomized study of two maintenance treatments: (a) I6 patients received intermittent intrathecal doses of MTX plus DMT, and (b) I6 patients received intermittent...

journal_title：British journal of haematology

authors： Muriel FS,Schere D,Barengols A,Eppinger-Helft M,Braier JL,Pavlovsky S,Macchi GH,Guman L

更新日期：1976-09-01 00:00:00

abstract：:S-Methylation by thiopurine methyltransferase (TPMT) is an important route of metabolism for the thiopurine drugs. About one in 300 individuals are homozygous for a TPMT mutation associated with very low enzyme activity and severe myelosuppression if treated with standard doses of drug. To validate the use of molecula...

journal_title：British journal of haematology

authors： Coulthard SA,Rabello C,Robson J,Howell C,Minto L,Middleton PG,Gandhi MK,Jackson G,McLelland J,O'Brien H,Smith S,Reid MM,Pearson AD,Hall AG

更新日期：2000-09-01 00:00:00

abstract：:A new, in-line high-efficiency 3-5 log10 leucodepletion filter system (Leukotrap RC system) was used to investigate the effect of pre-storage white cell removal on the quality of AS-3 red cell concentrates stored for 42 d at 4 degrees C. Median residual white cell content was 4 x 10(5) when filtration was performed at...

journal_title：British journal of haematology

authors： Heaton WA,Holme S,Smith K,Brecher ME,Pineda A,AuBuchon JP,Nelson E

更新日期：1994-06-01 00:00:00

abstract：:The thiol isomerase enzymes protein disulphide isomerase (PDI) and endoplasmic reticulum protein 5 (ERp5) are released by resting and activated platelets. These re-associate with the cell surface where they modulate a range of platelet responses including adhesion, secretion and aggregation. Recent studies suggest the...

journal_title：British journal of haematology

authors： Holbrook LM,Watkins NA,Simmonds AD,Jones CI,Ouwehand WH,Gibbins JM

更新日期：2010-02-01 00:00:00

abstract：:We have characterized the molecular defect in two families with severe factor VII (FVII) deficiency. In family I, the proband was found to be homozygous for a novel 18 bp deletion in exon 8 (g.10896-10913del) resulting in the in-frame deletion of six amino acids in the serine protease domain. Molecular modelling sugge...

journal_title：British journal of haematology

authors： Gomez K,Laffan MA,Kemball-Cook G,Pasi J,Layton M,Singer JD,Tuddenham EG,McVey JH

更新日期：2004-07-01 00:00:00

abstract：:It is the capacity to generate thrombin, and the enzymatic work that thrombin does, that determines blood coagulability. Therefore, measurement of the enzymatic work potential of thrombin provides a method for quantifying the composite effect of the multiple factors that determine coagulation capacity. The application...

journal_title：British journal of haematology

authors： Baglin T

更新日期：2005-09-01 00:00:00

abstract：:Although haematopoietic cell transplantation (HCT) is curative for sickle cell anaemia (SCA), concerns about its short- and long-term toxicities limit its application. A potential toxicity is an adverse effect on growth. To identify an HCT growth effect, serial height and weight measurements from 53 children and adole...

journal_title：British journal of haematology

authors： Eggleston B,Patience M,Edwards S,Adamkiewicz T,Buchanan GR,Davies SC,Dickerhoff R,Donfield S,Feig SA,Giller RH,Haight A,Horan J,Hsu LL,Kamani N,Lane P,Levine JE,Margolis D,Moore TB,Ohene-Frempong K,Redding-Lallinger

更新日期：2007-02-01 00:00:00

abstract：:The sera of 14 patients with quinine/quinidine-dependent thrombocytopenia were studied in the platelet suspension immunofluorescence test (PSIFT), the 51Cr-lysis assay and the complement fixation test (CFT). When anti-Ig or anti-IgG reagents were used, the PSIFT proved to be a little more sensitive than the 51Cr-lysis...

journal_title：British journal of haematology

authors： van Leeuwen EF,Engelfriet CP,von dem Borne AE

更新日期：1982-08-01 00:00:00

abstract：:Risk assessment for patients with sickle cell disease (SCD) remains challenging as it depends on an individual physician's experience and ability to integrate a variety of test results. We aimed to provide a new risk score that combines clinical, laboratory, and imaging data. In a prospective cohort of 600 adult patie...

journal_title：British journal of haematology

authors： Sachdev V,Tian X,Gu Y,Nichols J,Sidenko S,Li W,Beri A,Layne WA,Allen D,Wu CO,Thein SL

更新日期：2021-01-28 00:00:00

abstract：:The response to 0.8 ml of D-positive (ccDEE) red cells injected i.v., together with 1 microgram anti-D i.m., was studied in 13 D-negative male volunteers (test subjects); the red cells were labelled with 51Cr and were found to have a mean survival time of 24 d, after correction for Cr elution. Within the following 5 m...

journal_title：British journal of haematology

authors： Contreras M,Mollison PL

更新日期：1983-01-01 00:00:00

abstract：:Inhibition of apoptosis (genetically programmed active cell death) by p210 BCR-ABL expression is a mechanism that might contribute to clonal expansion in chronic myeloid leukaemia (CML). Since cell death following exposure to ionizing radiation and many chemotherapeutic agents can occur by the apoptotic pathway, inhib...

journal_title：British journal of haematology

authors： Amos TA,Lewis JL,Grand FH,Gooding RP,Goldman JM,Gordon MY

更新日期：1995-10-01 00:00:00

abstract：:The surface markers of immunoglobulin secreting cells (ISC) in bone marrow and peripheral blood were analysed. Circulating ISC bear surface Ig and Ia-like antigens. However, these markers were not detectable on ISC in bone marrow. Fc and complement receptors were not present on circulating ISC. The areas of plaques co...

journal_title：British journal of haematology

authors： Gyotoku Y,Mori M,Nonaka Y,Nagata Y,Saito Y

更新日期：1985-04-01 00:00:00

abstract：:Peripheral blood progenitor cell (PBPC) mobilization was evaluated in 53 patients receiving the high-dose sequential (HDS) regimen: 27 had non-Hodgkin's lymphoma or Hodgkin's disease, primary refractory or at first relapse, 26 had non-Hodgkin's lymphoma at diagnosis. Mobilization was assessed following either 7 g/m2 c...

journal_title：British journal of haematology

authors： Tarella C,Castellino C,Cherasco C,Bondesan P,Giaretta F,Corradini P,Caracciolo D,Gavarotti P,Pileri A

更新日期：1997-10-01 00:00:00