Recent advances in the pathogenesis and treatment of juvenile myelomonocytic leukaemia.

abstract：:We studied the activity and stage of chronic liver disease in 45 HCV-seropositive/HIV-seronegative patients with severe haemophilia followed for at least 10 years. HCV-RNA was detected in serum in 36 patients (80%) Viraemic cases were further analysed for HCV genotypes: 10 (28%) were infected by type 1a, 10 (28%) by t...

journal_title：British journal of haematology

authors： Tagariello G,Pontisso P,Davoli PG,Ruvoletto MG,Traldi A,Alberti A

更新日期：1995-11-01 00:00:00

abstract：:Platelets have recently been shown to trigger intrinsic coagulation by two alternative pathways, protect active clotting factors from inactivation by plasma inhibitors and catalyse intrinsic coagulation reactions on the platelet surface to form fibrin. To determine whether these platelet coagulant activities (PCA) mig...

journal_title：British journal of haematology

authors： Walsh PN,Rogers PH,Marder VJ,Gagnatelli G,Escovitz ES,Sherry S

更新日期：1976-03-01 00:00:00

abstract：:Patients belonging to four families with 'atypical elliptocytosis' have been investigated. Clinical, haematological, erythrokinetic and enzymatic characteristics as well as the effect of splenectomy are discussed. These studies appear to define the fundamental features of a particular disorder or a variety of heredita...

journal_title：British journal of haematology

authors： Torlontano G,Fioritoni G,Salvati AM

更新日期：1979-11-01 00:00:00

abstract：:The regulation of granulocyte-colony stimulating factor (G-CSF) and interleukin-6 (IL-6) mRNA was studied in human adherent monocytes in response to the protein kinase C activator, oleolyl-acetylglycerol (OAG), the calcium-ionophore A23187 and the cyclic AMP elevating agents, dibutyryl c-AMP (DBcAMP), cholera toxin an...

journal_title：British journal of haematology

authors： Vellenga E,van der Vinne B,De Wolf JT,Halie MR

更新日期：1991-05-01 00:00:00

abstract：:A uniform system of classification and nomenclature of the acute leukaemias, at present lacking, should permit more accurate recording of the distribution of cases entered into clinical trials, and could provide a reference standard when newly developed cell-surface markers believed to characterize specific cell types...

journal_title：British journal of haematology

authors： Bennett JM,Catovsky D,Daniel MT,Flandrin G,Galton DA,Gralnick HR,Sultan C

更新日期：1976-08-01 00:00:00

abstract：:To determine whether human red cells contain a proteolytic system capable of rapidly degrading unstable proteins, the fate of pulse-labelled puromycyl polypeptides was investigated. In erythroid bone marrow cells these unstable polypeptides were degraded to TCA-soluble fragments with a mean half-life of 4 . 5 min. How...

journal_title：British journal of haematology

authors： Vissers MC,Winterbourn CC,Carrell RW

更新日期：1983-03-01 00:00:00

abstract：:The outcomes of adult patients transplanted for Fanconi anaemia (FA) have not been well described. We retrospectively analysed 199 adult patients with FA transplanted between 1991 and 2014. Patients were a median of 16 years of age when diagnosed with FA, and underwent transplantation at a median age of 23 years. Time...

journal_title：British journal of haematology

authors： Bierings M,Bonfim C,Peffault De Latour R,Aljurf M,Mehta PA,Knol C,Boulad F,Tbakhi A,Esquirol A,McQuaker G,Sucak GA,Othman TB,Halkes CJM,Carpenter B,Niederwieser D,Zecca M,Kröger N,Michallet M,Risitano AM,Ehninger G

更新日期：2018-01-01 00:00:00

abstract：:18F-fluoro-deoxyglucose (FDG) positron emission tomography (PET) might be a better tool than computerized tomography (CT) in predicting long-term treatment outcome in patients with relapsed chemosensitive lymphoma who are candidates for autologous stem cell transplantation (ASCT). We studied patients with recurrent or...

journal_title：British journal of haematology

authors： Schot B,van Imhoff G,Pruim J,Sluiter W,Vaalburg W,Vellenga E

更新日期：2003-10-01 00:00:00

abstract：:To date, there is a lack of long-term safety and efficacy data for iron chelation therapy in transfusion-dependent patients with sickle cell disease (SCD). To evaluate the long-term safety and efficacy of deferasirox (a once-daily oral iron chelator), patients with SCD completing a 1-year, Phase II, randomized, defero...

journal_title：British journal of haematology

authors： Vichinsky E,Bernaudin F,Forni GL,Gardner R,Hassell K,Heeney MM,Inusa B,Kutlar A,Lane P,Mathias L,Porter J,Tebbi C,Wilson F,Griffel L,Deng W,Giannone V,Coates T

更新日期：2011-08-01 00:00:00

abstract：:Both atypical haemolytic uraemic syndrome (aHUS) and the HELLP syndrome (haemolytic anaemia, elevated liver enzymes, and low platelets) are thrombotic microangiopathies characterized by microvascular endothelial activation, cell injury and thrombosis. aHUS is a disease of complement dysregulation, specifically a gain ...

journal_title：British journal of haematology

authors： Fang CJ,Richards A,Liszewski MK,Kavanagh D,Atkinson JP

更新日期：2008-11-01 00:00:00

abstract：:The continued improvement in the prognosis of childhood acute myeloid leukaemia (AML) has been paralleled by the use of increasingly intensive therapy. This has led to attempts to develop risk-directed strategies in which the most intensive treatment is reserved for those at highest risk of relapse. Unfortunately, cur...

journal_title：British journal of haematology

authors： Goulden N,Virgo P,Grimwade D

更新日期：2006-08-01 00:00:00

abstract：:Ibrutinib is associated with durable responses in patients with Waldenström macroglobulinaemia (WM). We hypothesized that response depth is predictive of progression-free survival (PFS) in WM patients treated with ibrutinib. Using landmark analyses, we evaluated response depth in two cohorts of WM patients treated wit...

journal_title：British journal of haematology

authors： Castillo JJ,Abeykoon JP,Gustine JN,Zanwar S,Mein K,Flynn CA,Demos MG,Guerrera ML,Kofides A,Liu X,Munshi M,Tsakmaklis N,King R,Yang G,Hunter ZR,Advani RH,Palomba ML,Ansell SM,Gertz MA,Kapoor P,Treon SP

更新日期：2020-11-18 00:00:00

abstract：:The effect of parenteral administration of IL-11 on gastrointestinal iron absorption was evaluated. A significant increase in the absorption of 59Fe-tagged food iron fed to fasting rats was observed when two subcutaneous injections of IL-11 were given 48 and 24 h prior to testing. Relatively similar increases of 25% w...

journal_title：British journal of haematology

authors： Baynes RD,Cook JD,Keith J

更新日期：1995-09-01 00:00:00

abstract：:Chronic lymphocytic leukaemia/small lymphocytic lymphoma (CLL) is one of the more common forms of B cell malignancy. Although the condition has a variable clinical course, the trend is towards eventual relapse and the disease is considered incurable. Whilst the majority of the circulating CD5-positive neoplastic B cel...

journal_title：British journal of haematology

authors： Ramsay AD,Rodriguez-Justo M

更新日期：2013-07-01 00:00:00

abstract：:We treated 21 elderly patients with high-risk myelodysplasia (n = 14) or secondary acute myeloid leukaemia (n = 7) with 2 mg of melphalan orally once a day until a complete peripheral response was obtained or until there was evidence of treatment failure. We observed seven (30%) complete and two (10%) partial peripher...

journal_title：British journal of haematology

authors： Denzlinger C,Bowen D,Benz D,Gelly K,Brugger W,Kanz L

更新日期：2000-01-01 00:00:00

abstract：:Platelet associated C3c and C3d (PAC3c and PAC3d) were quantitated by enzyme linked assay in 105 patients with idiopathic autoimmune thrombocytopenia (AITP) in whom elevated platelet associated immunoglobulins (IgG and IgM) had previously been documented. Increased levels of complement components were demonstrated in ...

journal_title：British journal of haematology

authors： Hegde UM,Bowes A,Roter BL

更新日期：1985-05-01 00:00:00

abstract：:We have investigated the kinetics of colony formation by progenitor cells in chronic myeloid leukaemia (CML) using erythroid burst-forming units (BFU-E) as a model system. For this, we scored the numbers of subcolonies produced by individual BFU-E in cultures of normal marrow and blood cells and in cultures of CML blo...

journal_title：British journal of haematology

authors： Marley SB,Lewis JL,Goldman JM,Gordon MY

更新日期：1996-06-01 00:00:00

abstract：:We report the clinical outcome and results of chimaerism analysis in various cell lineages of 30 patients given non-myeloablative conditioning, followed by allogeneic stem cell transplantation (SCT). The commonest diagnoses were chronic myelogenous leukaemia (n = 11) and solid tumours (n = 11). Twenty-one patients rec...

journal_title：British journal of haematology

authors： Mattsson J,Uzunel M,Brune M,Hentschke P,Barkholt L,Stierner U,Aschan J,Ringdén O

更新日期：2001-12-01 00:00:00

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

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

abstract：:Persistent lymphocytosis is usually associated with a malignant lymphoproliferative disease (MLPD). We report six female patients presenting a chronic, moderate lymphocytosis of 2-16 years duration with atypical binucleated lymphocytes on peripheral blood smears. Further investigation showed a polyclonal increase in s...

journal_title：British journal of haematology

authors： Troussard X,Valensi F,Debert C,Maynadie M,Schillinger F,Bonnet P,Macintyre EA,Flandrin G

更新日期：1994-10-01 00:00:00

abstract：:Failed surface expression of the complement decay-accelerating factor (DAF) due to mutation of the PIG-A gene is a hallmark of affected paroxysmal nocturnal haemoglobinuria (PNH) blood elements. Previous findings that acute myelogenous leukaemia (AML) blasts evolving in a PNH patient differed from idiopathic AML blast...

journal_title：British journal of haematology

authors： Stafford HA,Nagarajan S,Weinberg JB,Medof ME

更新日期：1995-01-01 00:00:00

abstract：:The transport of iron through erythroid cell membrane was studied in a model system, measuring ferrous iron uptake by reticulocytes. It was found that these cells were able to take up ferrous iron and to incorporate it into haem at a rate similar to that observed when diferric transferrin was the iron donor. No compar...

journal_title：British journal of haematology

authors： Egyed A

更新日期：1988-04-01 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：:Cooley's original description of beta-thalassaemia major included marked bone deformities as a characteristic feature. These were thought to be due to expansion of haemopoiesis attempting to compensate for the congenital anaemia. Regular blood transfusions from infancy prevents these skeletal problems. Nevertheless, s...

journal_title：British journal of haematology

authors： Jensen CE,Tuck SM,Agnew JE,Koneru S,Morris RW,Yardumian A,Prescott E,Hoffbrand AV,Wonke B

更新日期：1998-12-01 00:00:00

abstract：:The molecular basis of severe type I factor (F)VII deficiency was investigated in two Algerian patients. One patient, a 13-year-old-girl who has suffered from severe bleeding since birth, was homozygous for a 7-bp deletion (nt 7774-7780) and a 251-bp insertion (nt 7773-7781) of mitochondrial origin, in IVS 4 acceptor ...

journal_title：British journal of haematology

authors： Borensztajn K,Chafa O,Alhenc-Gelas M,Salha S,Reghis A,Fischer AM,Tapon-Bretaudière J

更新日期：2002-04-01 00:00:00

abstract：:Idiopathic thrombotic thrombocytopenic purpura (TTP) is characterized by frequent recurrences. Effective screening for relapses will enable intervention prior to overt episodes of TTP. The present study used a modified assay to detect ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 motif, 13) ac...

journal_title：British journal of haematology

authors： Jin M,Casper TC,Cataland SR,Kennedy MS,Lin S,Li YJ,Wu HM

更新日期：2008-05-01 00:00:00

abstract：:Peripheral blood levels of BFU-e, CFU-GM and CFU-mix were studied serially in nine patients with acute leukaemia in remission during the period of recovery that followed induction or consolidation chemotherapy. Following 23 courses of treatment in the nine patients, the values for all three classes of progenitor were ...

journal_title：British journal of haematology

authors： Reid CD,Kirk A,Muir J,Chanarin I

更新日期：1989-05-01 00:00:00

abstract：:Inhibitors of heat-shockprotein 90 (Hsp90) have been proposed as a novel therapeutic option for Chronic Lymphocytic Leukaemia (CLL), particularly as their mechanism of action appears independent of mutations of ATM or TP53. We investigated the activity of a novel Hsp90 inhibitor, SNX7081, against a panel of eight haem...

journal_title：British journal of haematology

authors： Best OG,Singh N,Forsyth C,Mulligan SP

更新日期：2010-10-01 00:00:00

abstract：:Pituitary tumour transforming gene (PTTG) isolated from pituitary tumour cells transforms cells in vitro and causes in vivo tumour formation. PTTG is expressed in several human tumours and cell lines. In normal adult tissues, the testis expresses abundant levels of PTTG mRNA comparable to that found in tumour cells. A...

journal_title：British journal of haematology

authors： Stoika R,Melmed S

更新日期：2002-12-01 00:00:00

abstract：:Comparative genomic hybridization (CGH) was used to analyse 34 follicular lymphoma (FL) samples. 27 samples showed DNA sequence copy number changes of at least one genomic region (26 samples with at least one gain and nine with at least one loss). Some chromosomes or chromosomal regions were preferentially involved. T...

journal_title：British journal of haematology

authors： Avet-Loiseau H,Vigier M,Moreau A,Mellerin MP,Gaillard F,Harousseau JL,Bataille R,Milpied N

更新日期：1997-04-01 00:00:00