Haemophilia B Leyden arising de novo by point mutation in the putative factor IX promoter region.

abstract：:To clarify the mechanisms underlying thrombocytosis secondary to infections, we longitudinally studied serum levels of thrombopoietin (TPO) and interleukin (IL)-6 in 15 infants and young children with prominent thrombocytosis (platelets >700 x 10(9)/l) following acute infections and 116 age-matched controls using an e...

journal_title：British journal of haematology

authors： Ishiguro A,Suzuki Y,Mito M,Shimbo T,Matsubara K,Kato T,Miyazaki H

更新日期：2002-03-01 00:00:00

abstract：:Tissue factor pathway inhibitor (TFPI) is released to circulating blood after intravenous (i.v.) and subcutaneous (s.c.) injections of heparins, and may thus contribute to the antithrombotic effect of heparins. We have recently shown that total TFPI activity, plasma free TFPI antigen, and heparin releasable TFPI were ...

journal_title：British journal of haematology

authors： Bendz B,Hansen JB,Andersen TO,Ostergaard P,Sandset PM

更新日期：1999-12-01 00:00:00

abstract：:Multiple myeloma cell lines express functional receptors for insulin-like growth factors (IGFs) and several cell types that make up the bone marrow microenvironment produce these cytokines. This suggests that IGFs may play a role in survival and/or expansion of the malignant clone within the marrow in patients with mu...

journal_title：British journal of haematology

authors： Xu F,Gardner A,Tu Y,Michl P,Prager D,Lichtenstein A

更新日期：1997-05-01 00:00:00

abstract：:Penicillin-treated human red blood cells (RBC) were lysed by the cooperation of autologous nonsensitized peripheral blood mononuclear cells and human anti-penicillin serum. Using a rapid (3 h) assay of antibody-dependent cell-mediated cytotoxicity (ADCC), lysis was proportional to serum (anti-penicillin antibody) conc...

journal_title：British journal of haematology

authors： Yust I,Frisch B,Goldsher N

更新日期：1981-03-01 00:00:00

abstract：:We studied MDS-associated inhibitory activity, which inhibited colony formation in vitro of granulocyte-macrophage progenitors (CFU-GM). Macrophages obtained from MDS bone marrow mononuclear cells (BM-MNC) when pretreated with granulocyte-macrophage colony stimulating factor (GM-CSF) suppressed the growth of normal CF...

journal_title：British journal of haematology

authors： Ohmori M,Ohmori S,Ueda Y,Tohyama K,Yoshida Y,Uchino H

更新日期：1990-02-01 00:00:00

abstract：:Peripheral blood stem cell autografts for the treatment of chronic myeloid leukaemia (CML) are currently under evaluation. A patient with CML received intensive chemotherapy followed by granulocyte colony-stimulating factor prior to the collection of peripheral blood derived stem cells. He developed unusually severe, ...

journal_title：British journal of haematology

authors： Clark RE,Lee ES

更新日期：1995-06-01 00:00:00

abstract：:There are considerable differences in haemopoietic activity between young children and adults on the one hand, and between adults and the elderly on the other. A fundamental unanswered question is whether these differences relate to discrete stages or are part of a continuous process. We have sought to define aspects ...

journal_title：British journal of haematology

authors： Marley SB,Lewis JL,Davidson RJ,Roberts IA,Dokal I,Goldman JM,Gordon MY

更新日期：1999-07-01 00:00:00

abstract：:Notwithstanding the improvement in treatment results for paediatric T cell acute lymphoblastic leukaemia (T-ALL) it remains important to understand if genetic aberrations influence therapy response. PTEN tumour suppressor gene inactivation is a frequent event in T-ALL but its effect on patient therapy response is deba...

journal_title：British journal of haematology

authors： Paganin M,Grillo MF,Silvestri D,Scapinello G,Buldini B,Cazzaniga G,Biondi A,Valsecchi MG,Conter V,Te Kronnie G,Basso G

更新日期：2018-09-01 00:00:00

abstract：:Factor V Leiden (FVL)-carrying relatives of selected patients with venous thromboembolism (VTE) have much higher venous thrombotic risks than FVL-carrying relatives of unselected consecutive patients with VTE. To find an explanation for this, we explored other risk factors of VTE, in particular the presence of high fa...

journal_title：British journal of haematology

authors： Lensen R,Bertina RM,Vandenbroucke JP,Rosendaal FR

更新日期：2001-08-01 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

abstract：:Interferon(rIFN)-alpha, a successful therapeutic agent in the control of thrombocytosis, has been shown to suppress human megakaryopoiesis. We investigated bone marrow biopsies from 12 patients with thrombocytosis due to chronic myeloproliferative disorders. Prior to treatment as well as during rIFN-alpha-2c therapy, ...

journal_title：British journal of haematology

authors： Chott A,Gisslinger H,Thiele J,Fritz E,Linkesch W,Radaszkiewicz T,Ludwig H

更新日期：1990-01-01 00:00:00

abstract：:We have investigated the control of lysozyme gene expression in HL-60 cells induced to differentiate into macrophage-like cells with phorbol myristate acetate (PMA). Differentiation, as evidenced by cellular adherence, and morphological changes corresponded temporally to an increase in nonspecific esterase activity. T...

journal_title：British journal of haematology

authors： Polansky DA,Yang A,Brader K,Miller DM

更新日期：1985-05-01 00:00:00

abstract：:Phenotypic change of blood-type eosinophils to tissue-type eosinophils is induced by various cytokines. We examined the effect of nerve growth factor (NGF) as a candidate for a constitutive cytokine which is able to induce the phenotypic change of eosinophils. The viability of human peripheral blood eosinophils cultur...

journal_title：British journal of haematology

authors： Hamada A,Watanabe N,Ohtomo H,Matsuda H

更新日期：1996-05-01 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：:The chemotaxis of human malignant plasma cells is promoted by two extracellular matrix proteins (ECMs): fibronectin (FN) and laminin (LN). We examined the effect of the supernatant from a bone marrow stroma cell line, KM-101, on the chemotaxis of human malignant plasma cell lines to assess the chemotaxis-regulatory ro...

journal_title：British journal of haematology

authors： Shibayama H,Tagawa S,Hattori H,Harigaya K,Taga T,Machii T,Kitani T

更新日期：1996-06-01 00:00:00

abstract：:A digital image processing technique has been used to quantitate bone marrow fibre in histological material (both biopsy and necropsy) from 17 cases of myelofibrosis. An attempt was made to correlate the results of these measurements with other diagnostically relevant parameters. Bone marrow fibre was found to correla...

journal_title：British journal of haematology

authors： Bentley SA,Herman CJ

更新日期：1979-05-01 00:00:00

abstract：:This study describes the effect of ferritin on lymphocyte function in vitro. Peripheral blood lymphocytes isolated from normal donors were incubated with purified human splenic ferritin, and the mitogenic effect of phytohaemagglutinin (PHA), concanavalin A (Con A), pokeweed mitogen (PWM) and mixed lymphocyte reaction ...

journal_title：British journal of haematology

authors： Matzner Y,Hershko C,Polliack A,Konijn AM,Izak G

更新日期：1979-07-01 00:00:00

abstract：:Pegfilgrastim is a pegylated form of the granulocyte-colony stimulating factor, filgrastim. Herein, we report the results of a multicentre, randomized, double-blind phase III trial comparing the efficacy and safety of pegfilgrastim with filgrastim in patients with malignant lymphoma. Patients were randomized to receiv...

journal_title：British journal of haematology

authors： Kubo K,Miyazaki Y,Murayama T,Shimazaki R,Usui N,Urabe A,Hotta T,Tamura K

更新日期：2016-08-01 00:00:00

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 pattern of distribution of von Willebrand factor (VWF) in relatively large sheets of rat aortic endothelial cells (EC) obtained by the Häutchen technique were analysed by immunocytochemistry and light microscopy. EC were examined pre and post administration of a procoagulant mixture of factor Xa (F.Xa) and phospho...

journal_title：British journal of haematology

authors： Senis YA,Richardson M,Tinlin S,Maurice DH,Giles AR

更新日期：1996-04-01 00:00:00

abstract：:Over the last few years there has been rapid and radical change in the way clinical research in the UK is funded and supported within the NHS. This has resulted from restructuring and major new investment in research infrastructure, co-ordinated through Clinical Local Research Networks (CLRNs) and equivalent organisat...

journal_title：British journal of haematology

authors： Collins PW,Baglin TP,Dang R,Evans G,Greaves M,Laffan M,Pasi KJ,Rose P,Stanworth S,Toh CH

更新日期：2010-09-01 00:00:00

abstract：:Very Late Antigen-4 (VLA-4, α4β1-integrin, ITGA4) orchestrates cell-cell and cell-endothelium adhesion. Given the proposed role of VLA-4 in sickle cell disease (SCD) pathophysiology, we evaluated the ability of the VLA-4 blocking antibody natalizumab to inhibit SCD blood cell adhesion. Natalizumab recognized surface V...

journal_title：British journal of haematology

authors： White J,Krishnamoorthy S,Gupta D,Lancelot M,Moore N,Sarnaik S,Hobbs WE 2nd,Light DR,Hines P

更新日期：2016-09-01 00:00:00

abstract：:The role of serum macrophage inflammatory protein-1 alpha (MIP-1alpha) in bone disease and survival was evaluated in 85 newly diagnosed multiple myeloma (MM) patients. MIP-1alpha was elevated in MM patients and correlated with the extent of bone disease, bone resorption markers and levels of soluble receptor activator...

journal_title：British journal of haematology

authors： Terpos E,Politou M,Szydlo R,Goldman JM,Apperley JF,Rahemtulla A

更新日期：2003-10-01 00:00:00

abstract：:The findings of morphologically dysplastic features in haemopoietic cells in de novo acute myeloid leukaemia (AML) has been named AML with trilineage myelodysplasia (AML/TMDS). We analysed the clinical data, karyotypes, and treatment outcomes of 230 de novo AML patients treated with the Japan Adult Leukaemia Study Gro...

journal_title：British journal of haematology

authors： Kuriyama K,Tomonaga M,Matsuo T,Kobayashi T,Miwa H,Shirakawa S,Tanimoto M,Adachi K,Emi N,Hiraoka A

更新日期：1994-04-01 00:00:00

abstract：:A single immunoglobulin light chain lambda was identified in the blast cells of two out of 12 patients with common acute lymphoblastic leukaemia (C-ALL) using the alkaline phosphatase/anti-alkaline phosphatase (APAAP) technique. Inhibition at the cell surface proved that the reaction was a genuine anti-lambda reaction...

journal_title：British journal of haematology

authors： Markey GM,Alexander HD,Nolan RL,Morris TC,Robertson JH

更新日期：1989-01-01 00:00:00

abstract：:The heterogeneity of pyruvate kinase (PK) deficiency associated with hereditary haemolytic anaemia is emphasized by studies of a kindred harbouring two distinct mutant forms of this enzyme, both of which were kinetically defective with markedly decreased affinities for the substrate, phosphoenolypyruvate. The two isoe...

journal_title：British journal of haematology

authors： Pagila DE,Gray GR,Growe GH,Valentine WN

更新日期：1976-09-01 00:00:00

abstract：:The combined incidence of classical Philadelphia-negative myeloproliferative neoplasm (MPN) is 6-9/100 000 with a peak frequency between 50 and 70 years. MPN is less frequent in women of reproductive age. However, for essential thrombocythaemia (ET) in particular there is a second peak in women of reproductive age and...

journal_title：British journal of haematology

authors： Robinson SE,Harrison CN

更新日期：2020-05-01 00:00:00

abstract：:The Fanconi anaemia protein FANCD2 suppresses PPARƔ to maintain haematopoietic stem cell's (HSC) function; however, the underlying mechanism is not known. Here we show that FANCD2 acts in concert with the Notch target HES1 to suppress inflammation-induced PPARƔ in HSC maintenance. Loss of HES1 exacerbates FANCD2-KO HS...

journal_title：British journal of haematology

authors： Wu L,Li X,Lin Q,Chowdhury F,Mazumder MH,Du W

更新日期：2020-11-22 00:00:00

abstract：:In order to clarify the mechanism of the effect of erythropoietin (Epo) on the fetal haemoglobin (HbF) phenotype of peripheral erythrocytes, we studied the dose-response effect of Epo on HbF production by erythroid precursors derived from the peripheral blood of normal adult individuals and grown in a two-phase liquid...

journal_title：British journal of haematology

authors： Fibach E,Schechter AN,Noguchi CT,Rodgers GP

更新日期：1994-09-01 00:00:00

abstract：:The French-American-British (FAB) classification assigns patients with myelodysplastic syndromes to the category of refractory anaemia with excess blasts (RAEB) if they have a medullary blast count of 5-20%, and/or a peripheral blast count of 2-5%. The new World Health Organization (WHO) classification subdivides RAEB...

journal_title：British journal of haematology

authors： Germing U,Strupp C,Kuendgen A,Aivado M,Giagounidis A,Hildebrandt B,Aul C,Haas R,Gattermann N

更新日期：2006-01-01 00:00:00