Paediatric myelodysplastic syndromes and juvenile myelomonocytic leukaemia in the UK: a population-based study of incidence and survival.

abstract：:Shwachman-Diamond Syndrome (SDS) is a multi-system genetic disorder with bone marrow failure. SBDS, the gene associated with SDS, has been postulated to play a role in ribosome biogenesis and RNA processing, but its functions are still unknown. To study whether these pathways are interrupted when Sbds protein is lost,...

journal_title：British journal of haematology

authors： Rujkijyanont P,Adams SL,Beyene J,Dror Y

更新日期：2009-06-01 00:00:00

abstract：:Lymphoproliferative diseases of large granular lymphocytes (LDGL) may arise from either CD3+ T cells or CD3- natural killer (NK) cells. LDGL with clonal proliferation of large granular lymphocytes (LGL) is defined as LGL leukaemia. The number of patients with NK-LGL leukaemia reported is limited and the pathogenesis o...

journal_title：British journal of haematology

authors： Chou WC,Chiang IP,Tang JL,Su IJ,Huang SY,Chen YC,Liu MC,Lee FY,Wang CH,Shen MC,Chuang SM,Tien HF

更新日期：1998-12-01 00:00:00

abstract：:We present a Ph-positive chronic myeloid leukaemia patient who lost a complete cytogenetic response (CCR) of 23 months duration at the time of detection of a deletion, not previously observed, of chromosomes 9 and 22 sequences flanking the translocation breakpoint on the derivate 9 chromosome. To our knowledge, this i...

journal_title：British journal of haematology

authors： Arranz E,Gil-Fernández JJ,Ramiro S,Blas C,Renedo M,Alegre A,Escudero A,Fernández-Rañada JM

更新日期：2002-06-01 00:00:00

abstract：:The purpose of the present work were to identify the initial characteristics associated with long-term survival in chronic granulocytic leukaemia (CGL) and to analyse the accuracy of prognostic models in identifying long-term survivors. 813 Philadelphia (Ph) chromosome-positive, nonblastic CGL patients from six Americ...

journal_title：British journal of haematology

authors： Cervantes F,Robertson JE,Rozman C,Baccarani M,Tura S,Gómez GA,Braun TJ,Clarkson BD,Pereira A

更新日期：1994-06-01 00:00:00

abstract：:A multiparameter flow-cytometrical method for the quantitation of CD34(+)-cells present in adult human peripheral blood cells (PBMC) has been developed. PBMC from 13 healthy adult subjects were analysed for CD34(+)-cells by flow-cytometry, with only the lymphoid population of cells negative for anti-CD3-moAb included ...

journal_title：British journal of haematology

authors： Serke S,Säuberlich S,Huhn D

更新日期：1991-04-01 00:00:00

abstract：:Pleiotrophin (PTN), a tightly regulated angiogenic and mitogenic heparin-binding protein, is markedly elevated in a variety of aggressive solid tumours. The role of PTN in haematological malignancies, however, has not been previously evaluated. This study demonstrated that PTN serum levels were elevated in multiple my...

journal_title：British journal of haematology

authors： Yeh HS,Chen H,Manyak SJ,Swift RA,Campbell RA,Wang C,Li M,Lee HJ,Waterman G,Gordon MS,Ma J,Bonavida B,Berenson JR

更新日期：2006-06-01 00:00:00

abstract：:The changes in bone marrow (BM) stem cell reserve and function and stromal cell function in patients with active systemic lupus erythematosus (SLE) were investigated. The study was carried out on seven SLE patients and 28 healthy controls using flow cytometry and in vitro cell culture assays. We found that patients ha...

journal_title：British journal of haematology

authors： Papadaki HA,Boumpas DT,Gibson FM,Jayne DR,Axford JS,Gordon-Smith EC,Marsh JC,Eliopoulos GD

更新日期：2001-10-01 00:00:00

abstract：:In this paper we describe the placental transport of [3H]vitamin K1 in pregnant rats during the first 24 h after the oral administration of the vitamin. Vitamin K1 in the fetal livers ranged from 0.13% (3 h) to 2% (24 h) of the values found in the corresponding maternal livers. In spite of the low placental transfer o...

journal_title：British journal of haematology

authors： Hamulyák K,de Boer-van den Berg MA,Thijssen HH,Hemker HC,Vermeer C

更新日期：1987-03-01 00:00:00

abstract：:Serum antiplatelet IgG and platelet-associated IgG (PAIgG) were studied in 68 AIDS-free human immunodeficiency virus (HIV)-infected patients with severe immunologic thrombocytopenic purpura (ITP), for the presence of platelet autoantibodies. Serum IgG with antiplatelet activity was found in 72% of the sera. However, t...

journal_title：British journal of haematology

authors： Bettaieb A,Oksenhendler E,Fromont P,Duedari N,Bierling P

更新日期：1989-10-01 00:00:00

abstract：:New diagnostic criteria for the diagnosis of the various myelodysplastic syndromes (MDS) are proposed, and a detailed description is given of the features that may help define MDS. Five MDS are described: (1) refractory anaemia (RA), (2) RA with ring sideroblasts, (3) RA with excess of blasts (RAEB), (4) chronic myelo...

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：1982-06-01 00:00:00

abstract：:Haematological characteristics have been compared in 29 subjects with heterozygous beta0 thalassaemia and in 33 subjects with heterozygous beta+ thalassaemia, identified by the type of sickle cell-beta thalassaemia among close relatives, in a Jamaican Negro population. Total haemoglobin, MCV and MCH were significantly...

journal_title：British journal of haematology

authors： Millard DP,Mason K,Serjeant BE,Serjeant GR

更新日期：1977-06-01 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：:We studied erythrocyte calcium levels and uptake in a group of patients with sickle haemoglobinopathies of different clinical severity in an attempt to relate these measurements to the production of irreversibly sickled cells and disease severity. Erythrocyte calcium levels were measured by atomic absorption spectrosc...

journal_title：British journal of haematology

authors： Steinberg MH,Eaton JW,Berger E,Coleman MB,Oelshlegel FJ

更新日期：1978-12-01 00:00:00

abstract：:Bone disease in patients with thalassaemia major is a multifactorial and still poorly understood process. The present study evaluated 45 thalassaemic patients using dual X-ray absorptiometry at three sites (lumbar spine, head of femur and forearm) to assess bone mineral density, in parallel with a series of biochemica...

journal_title：British journal of haematology

authors： Voskaridou E,Kyrtsonis MC,Terpos E,Skordili M,Theodoropoulos I,Bergele A,Diamanti E,Kalovidouris A,Loutradi A,Loukopoulos D

更新日期：2001-01-01 00:00:00

abstract：:The dependence of survival time on a set of prognostic factors was explored by means of Cox's regression model in 137 cases of myelofibrosis with myeloid metaplasia (MMM). The following parameters recorded at diagnosis proved to be important independent indicators of a poor prognosis: a higher value for age, a lower v...

journal_title：British journal of haematology

authors： Barosi G,Berzuini C,Liberato LN,Costa A,Polino G,Ascari E

更新日期：1988-12-01 00:00:00

abstract：:We evaluated the blood from 150 patients with primary AL-amyloidosis for circulating monoclonal plasma cells using a sensitive slide-based immunofluorescence technique. The percentage of monoclonal blood plasma cells (BPC) that were in S-phase was determined by the bromodeoxyuridine labelling index (BLI). Monoclonal B...

journal_title：British journal of haematology

authors： McElroy EA Jr,Witzig TE,Gertz MA,Greipp PR,Kyle RA

更新日期：1998-02-01 00:00:00

abstract：:Until recently, primary central nervous system lymphoma (PCNSL) was associated with a uniformly dismal prognosis. It is now reasonable to anticipate long-term survival and possibly cure for a significant proportion of patients diagnosed with PCNSL. Accumulated data generated over the past 10 years has provided evidenc...

journal_title：British journal of haematology

authors： Wang CC,Carnevale J,Rubenstein JL

更新日期：2014-08-01 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：:Management of the acute painful crisis (APC) of sickle cell disease (SCD) remains unsatisfactory despite advances in the understanding and management of acute pain in other clinical settings. One reason for this is an unsophisticated approach to the use of opioid analgesics for pain management. This applies to haemato...

journal_title：British journal of haematology

authors： Telfer P,Bahal N,Lo A,Challands J

更新日期：2014-07-01 00:00:00

abstract：:Five out of nine adults (55%) with lymphoblastic disease developed severe avascular necrosis of bone (AVN) when treated with a Berlin-Frankfurt-Munster (BFM) ALL protocol similar to the current joint MRC-ECOG ALL trial (UKALL XII). The principal purpose of these intensified regimens is to improve long-term disease-fre...

journal_title：British journal of haematology

authors： Chan-Lam D,Prentice AG,Copplestone JA,Weston M,Williams M,Hutton CW

更新日期：1994-01-01 00:00:00

abstract：:Plasma and urine folate fractions were evaluated after ingestion of radioactive N5-methyl-tetrahydrofolic acid by a normal control (subject I), a patient on maintenance haemodialysis for chronic glomerulonephritis (subject 2), and an anephric patient on haemodialysis (subject 3). In subjects 1 and 2 maximal plasma rad...

journal_title：British journal of haematology

authors： Retief FP,Heyns AP,Oosthuizen M,Reenen OR

更新日期：1977-07-01 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：:Serum erythropoietin (Epo) was measured in 23 patients before, during and after intensive cytostatic treatment courses for acute leukaemia or before bone marrow transplantation. A marked increase was seen in all patients, starting 1 or 2 d after initiation of treatment. A peak was reached after about 7 d, at levels as...

journal_title：British journal of haematology

authors： Birgegård G,Wide L,Simonsson B

更新日期：1989-07-01 00:00:00

abstract：:The myelodysplastic syndromes (MDS) display both haematological and biological heterogeneity with variable leukaemia potential. MicroRNAs play an important role in tumour suppression and the regulation of self-renewal and differentiation of haematopoietic progenitors. Using a microarray platform, we evaluated microRNA...

journal_title：British journal of haematology

authors： Sokol L,Caceres G,Volinia S,Alder H,Nuovo GJ,Liu CG,McGraw K,Clark JA,Sigua CA,Chen DT,Moscinski L,Croce CM,List AF

更新日期：2011-04-01 00:00:00

abstract：:Thrombosis occurs in 37% of children with acute lymphoblastic leukaemia (ALL) and is related to an L-asparaginase-induced acquired antithrombin (AT) deficiency. The incidence dictates the need for anticoagulant prophylaxis. Direct thrombin inhibitors (DTI) are independent of AT for effect and may thus have advantages ...

journal_title：British journal of haematology

authors： Kuhle S,Lau A,Bajzar L,Vegh P,Halton J,Cherrick I,Anderson R,Desai S,McCusker P,Wu J,Abshire T,Mahoney D,Mitchell L

更新日期：2006-09-01 00:00:00

abstract：:The major pathways of glucose metabolism in the malaria parasite, Plasmodium falciparum, have now been elucidated, and the structures and properties of parasite-specific enzymes are presently being investigated. Little is known, however, about the enzymes catalysing monosaccharide interconversions in the parasite. In ...

journal_title：British journal of haematology

authors： Weidanz JA,Campbell P,Moore D,DeLucas LJ,Rodén L,Thompson JN,Vezza AC

更新日期：1996-12-01 00:00:00

abstract：:Paediatric recommendations for unfractionated heparin (UFH) management are extrapolated from adult trials, a practice that may contribute to the inferior UFH-related outcomes in children compared to adults. This is the first study to determine UFH concentration in a population of children and correlated UFH concentrat...

journal_title：British journal of haematology

authors： Newall F,Ignjatovic V,Johnston L,Summerhayes R,Lane G,Cranswick N,Monagle P

更新日期：2010-09-01 00:00:00

abstract：:Early T-cell precursor acute lymphoblastic leukaemia (ETP-ALL) is a recently identified subtype of T-ALL with distinctive gene expression and cell marker profiles, poor response to chemotherapy and a very high risk of relapse. We determined the reliability of restricted panel of cell markers to identify EPT-ALL using ...

journal_title：British journal of haematology

authors： Inukai T,Kiyokawa N,Campana D,Coustan-Smith E,Kikuchi A,Kobayashi M,Takahashi H,Koh K,Manabe A,Kumagai M,Ikuta K,Hayashi Y,Tsuchida M,Sugita K,Ohara A

更新日期：2012-02-01 00:00:00

abstract：:Therapy for a first episode of venous thromboembolism (VTE) typically includes a vitamin K antagonist, such as warfarin, for 3-6 months at an international normalized ratio (INR) of 2-3. After the cessation of warfarin therapy, unprovoked VTE is associated with a recurrence rate of 5-15% per year. Prolonging initial t...

journal_title：British journal of haematology

authors： Bauer KA

更新日期：2004-10-01 00:00:00

abstract：:It was previously shown that the abnormal surface characteristics and defective bactericidal function of polymorphonuclear leucocytes (PMN) in the Chediak-Higashi syndrome (CHS) are correlated with impaired microtubule assembly, and in one patient direct electron microscopic evidence for an anomaly in microtubule asse...

journal_title：British journal of haematology

authors： Boxer LA,Albertini DF,Baehner RL,Oliver JM

更新日期：1979-10-01 00:00:00