Langerhans cell histiocytosis in children: from the bench to bedside for an updated therapy.

abstract：:We examined the effect of vitamin D receptor (VDR) polymorphisms at exon 2 (FokI) and intron 8 (BsmI) on the stature and bone mineral density at femoral neck (FBMD) and lumbar spine (LBMD) in 108 prepubertal and pubertal homozygous beta thalassaemic patients, regularly treated. We found significantly shorter stature a...

journal_title：British journal of haematology

authors： Ferrara M,Matarese SM,Francese M,Borrelli B,Coppola A,Coppola L,Esposito L

更新日期：2002-05-01 00:00:00

abstract：:To investigate the role of neutrophils in complement-induced changes in vascular permeability, skin wheal and flare responses to intradermal injection of autologous activated serum complement were measured in normal and neutropenic subjects. In normal subjects, responses were dose-dependent and were abolished by remov...

journal_title：British journal of haematology

authors： Williamson LM,Sheppard K,Davies JM,Fletcher J

更新日期：1986-10-01 00:00:00

abstract：:Eleven patients are described in whom myelomatosis was complicated by the laboratory and clinical features of the hyperviscosity syndrome (HVS). The myeloma type was IgA in nine and IgG3 in two. In those patients with IgA myeloma the HVS was related to the presence of high molecular weight complexes in the serum. Remi...

journal_title：British journal of haematology

authors： Preston FE,Cooke KB,Foster ME,Winfield DA,Lee D

更新日期：1978-04-01 00:00:00

abstract：:A simplified method of International Normalized Ratio (INR) derivation using linear regression of certified INR plotted against local prothrombin time (PT) results has been compared with INR from conventional orthogonal regression. Linear regression assumes error only with the local PT results whereas orthogonal regre...

journal_title：British journal of haematology

authors： Poller L,Barrowcliffe TW,van den Besselaar AM,Jespersen J,Tripodi A,Houghton D

更新日期：1997-09-01 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：:We present evidence that alterations in marrow adherent cell (M phi) function may play a role in the suppression of erythropoiesis in some patients with fungal infection. Bone marrow (BM) cells from 12 normals and 10 patients with histoplasmosis were cultured in plasma clots before and after removal of M phi. BM from ...

journal_title：British journal of haematology

authors： Zanjani ED,McGlave PB,Davies SF,Banisadre M,Kaplan ME,Sarosi GA

更新日期：1982-03-01 00:00:00

abstract：:We investigated the effects of Il-12 on functional properties of CD3+ CD8+ granular lymphocytes (GL) of of patients with lymphoproliferative disease of granular lymphocytes (LDGL). To this aim, in 10 cases with clonal CD3+ GL proliferation (nine cases with an associated TCR alpha/beta receptor and one case with a TCR ...

journal_title：British journal of haematology

authors： Zambello R,Trentin L,Cassatella MA,Raimondi R,Cerutti A,Enthammer C,Facco M,Agostini C,Semenzato G

更新日期：1996-02-01 00:00:00

abstract：:The presence of autoimmune diseases, including Systemic Sclerosis (SSc), suggest failure of the normal immune regulatory processes leading to activation and expansion of autoreactive effector immune cells. Recently, stem cell transplantation emerged as a novel rescue therapy for a variety of refractory autoimmune dise...

journal_title：British journal of haematology

authors： Cipriani P,Ruscitti P,Giacomelli R

更新日期：2015-02-01 00:00:00

abstract：:The PFA-100 measures platelet plug formation under shear stress and is strongly dependent on von Willebrand Factor (VWF) levels in plasma. We therefore hypothesized that elevated VWF levels, possibly as a result of acute inflammation, adversely influence PFA-100 results. Healthy volunteers received either 2 ng/kg endo...

journal_title：British journal of haematology

authors： Homoncik M,Blann AD,Hollenstein U,Pernerstorfer T,Eichler HG,Jilma B

更新日期：2000-12-01 00:00:00

abstract：:Despite its' central role, the precise mechanisms of the phosphoinositide 3-kinase/Akt (PI3K)/Akt pathway activation in acute myeloid leukaemia (AML) have not been elucidated. Recently, a recurrent novel AKT1 pleckstrin homology domain (PHD) mutation leading to membrane translocation, constitutive AKT activation and l...

journal_title：British journal of haematology

authors： Tibes R,Kornblau SM,Qiu Y,Mousses SM,Robbins C,Moses T,Carpten JD

更新日期：2008-02-01 00:00:00

abstract：:Use of asparaginase (ASNase) in the treatment of relapsed childhood acute lymphoblastic leukaemia (ALL) is associated with a high rate of hypersensitive reactions. 'Silent' inactivation may additionally reduce treatment intensity. Therefore, PEG-ASNase (Oncaspar), a polyethylene glycol conjugate of the native Escheric...

journal_title：British journal of haematology

authors： Vieira Pinheiro JP,Müller HJ,Schwabe D,Gunkel M,Casimiro da Palma J,Henze G,von Schütz V,Winkelhorst M,Würthwein G,Boos J

更新日期：2001-04-01 00:00:00

abstract：:Myelodysplastic syndromes (MDS) and the mixed myelodysplastic/myeloproliferative disorder juvenile myelomonocytic leukaemia (JMML) are rare haematopoietic stem cell diseases in children. While MDS-initiating events remain largely obscure, a growing body of clinical, genetic and laboratory evidence suggests that JMML i...

journal_title：British journal of haematology

authors： Niemeyer CM,Kratz CP

更新日期：2008-03-01 00:00:00

abstract：:With the aim of producing unique targets for malignant cells we have identified peptide ligands for the clonal surface immunoglobulin isolated from the B cells of a chronic lymphocytic leukaemia (CLL) patient. The peptides were identified from random-peptide phage-display libraries. The obtained ligands bound specific...

journal_title：British journal of haematology

authors： Buhl L,Szecsi PB,Gisselø GG,Schafer-Nielsen C

更新日期：2002-03-01 00:00:00

abstract：:In the positron emission tomography (PET) era, traditional prognostic factors may not apply for patients with relapsed/refractory (R/R) diffuse large B cell lymphoma (DLBCL) undergoing autologous stem cell transplantation (ASCT). Moreover, little is known about prognostic factors in patients transplanted for transform...

journal_title：British journal of haematology

authors： Armand P,Welch S,Kim HT,LaCasce AS,Jacobsen ED,Davids MS,Jacobson C,Fisher DC,Brown JR,Coughlin E,Freedman AS,Chen YB

更新日期：2013-03-01 00:00:00

abstract：:Iron chelators are increasingly combined clinically but the optimal conditions for cellular iron mobilization and mechanisms of interaction are unclear. Speciation plots for iron(III) binding of paired combinations of the licensed iron chelators desferrioxamine (DFO), deferiprone (DFP) and deferasirox (DFX) suggest co...

journal_title：British journal of haematology

authors： Vlachodimitropoulou Koumoutsea E,Garbowski M,Porter J

更新日期：2015-09-01 00:00:00

abstract：:We tested four negative and two positive selection methods for separation of CD34+ cells from mobilized blood cells, and analysed fold-enrichment, purity and recovery of CD34+ cells after selection procedures. The elimination of mature CD34- cells was achieved by adhesion to nylon-wool fibre (5.9 +/- 1.0 mean fold-enr...

journal_title：British journal of haematology

authors： Di Nicola M,Bregni M,Siena S,Ruffini PA,Milanesi M,Ravagnani F,Gianni AM

更新日期：1996-09-01 00:00:00

abstract：:This is an historical account of the randomised trials in chronic lymphocytic leukaemia in the UK between the years 1978 to 2004, describing their gestation, the treatments used and the main lessons learnt. Those lessons include: (1) how best to use chlorambucil, which was the first effective treatment for CLL; (2) th...

journal_title：British journal of haematology

authors： Catovsky D,Else M

更新日期：2020-11-01 00:00:00

abstract：:This study assessed the relevance of 2013 European LeukaemiaNet (ELN) response categories on patients treated with common frontline tyrosine kinase inhibitors (TKI) in chronic myeloid leukaemia in chronic phase (CML-CP). Four hundred and eighty-seven patients treated with imatinib (400 mg; IM 400, n = 70; 800 mg; IM80...

journal_title：British journal of haematology

authors： Jain P,Kantarjian H,Sasaki K,Jabbour E,Dasarathula J,Nogueras Gonzalez G,Verstovsek S,Borthakur G,Wierda W,Kadia T,Dellasala S,Pierce S,Ravandi F,O'Brien S,Cortes J

更新日期：2016-04-01 00:00:00

abstract：:A female patient with delayed haemolytic transfusion reaction due to anti-M antibody is described. Diagnosis was based on laboratory evidence of haemolysis and on characteristic serological findings. Anti-M was detected in the recipient's serum 7 d after the last transfusion episode. This alloantibody had not been pre...

journal_title：British journal of haematology

authors： Sancho JM,Pujol M,Fernández F,Soler M,Manzano P,Feliu E

更新日期：1998-10-01 00:00:00

abstract：:The Complement 1 trial investigated the efficacy and safety of ofatumumab + chlorambucil with chlorambucil monotherapy in patients with previously untreated chronic lymphocytic leukaemia (CLL). On long-term follow-up in the chemoimmunotherapy arm vs. the chemotherapy arm there was an estimated 12% (not significant) an...

journal_title：British journal of haematology

authors： Offner F,Robak T,Janssens A,Govind Babu K,Kloczko J,Grosicki S,Mayer J,Panagiotidis P,Schuh A,Pettitt A,Montillo M,Werner O,Vincent G,Khanna S,Hillmen P

更新日期：2020-09-01 00:00:00

abstract：:Relapses involving the central nervous system (CNS) are rare in children and adolescents with ALK+ anaplastic large cell lymphoma (ALCL) treated with regimens including CNS prophylaxis. Early identification of patients at high-risk for CNS relapse would enable stratification and better adaptation of initial treatment ...

journal_title：British journal of haematology

authors： Del Baldo G,Abbas R,Woessmann W,Horibe K,Pillon M,Burke A,Beishuizen A,Rigaud C,Le Deley MC,Lamant L,Brugières L

更新日期：2020-07-09 00:00:00

abstract：:Protein S is expressed in a number of tissue types, one of the most physiologically relevant being the liver. However, transcriptional control of protein S gene expression is poorly understood. We have characterised a 638 bp area in the 5' flanking region of the human protein S gene, spanning all 10 previously reporte...

journal_title：British journal of haematology

authors： Hall AJ,Peake IR,Winship PR

更新日期：2006-11-01 00:00:00

abstract：:Two patients with acute immune haemolytic anaemia caused by diclofenac are described. Both patients had developed IgG drug-independent autoantibodies and drug-dependent antibodies. The drug-dependent antibodies in one patient reacted with red blood cells (RBC) only in the presence of urine from patients receiving dicl...

journal_title：British journal of haematology

authors： Salama A,Göttsche B,Mueller-Eckhardt C

更新日期：1991-04-01 00:00:00

abstract：:Analysis of complementary RNA molecules of junctional regions of rearranged T-cell-receptor-gamma genes show a pattern of conformational polymorphisms which is specific for an individual lymphocytic clone. In a blinded study we analysed formalin-fixed, paraffin-embedded histological specimens from gastrointestinal lym...

journal_title：British journal of haematology

authors： Koch OM,Probst M,Tiemann M,Jakob I,Volkenandt M,Wienecke R,Buer J,Atzpodien J,Lenz HJ,Danenberg PV

更新日期：1994-02-01 00:00:00

abstract：:Fanconi anaemia (FA) is an autosomal recessive and X-linked disease characterized by severe genetic instability and increased incidence of cancer. One explanation for this instability may be the cellular hypersensitivity to oxidative stress leading to chromosomal breaks. This study explored the possible oxidative dama...

journal_title：British journal of haematology

authors： Uziel O,Reshef H,Ravid A,Fabian I,Halperin D,Ram R,Bakhanashvili M,Nordenberg J,Lahav M

更新日期：2008-07-01 00:00:00

abstract：:Clusters and chains of basophilic micro-organisms were found on the red cells of a woman who suffered from malignant melanoma. The infection was clinically silent for at least 7 years. During her last year of life the patient became febrile and anaemic. Her spleen enlarged and the basophilic bodies on her red cells be...

journal_title：British journal of haematology

authors： Clark KG

更新日期：1975-02-01 00:00:00

abstract：:There are no accepted methods to predict the development of platelet transfusion refractoriness (PTR) due to human leucocyte antigen (HLA)-alloimmunization. Hence, matched platelets are usually given only to patients demonstrating PTR, necessarily resulting in some ineffective random donor platelets (RDPLT) transfusio...

journal_title：British journal of haematology

authors： Beligaswatte A,Tsiopelas E,Humphreys I,Bennett G,Robinson K,Davis K,Bardy P

更新日期：2013-08-01 00:00:00

abstract：:Nine patients with Fanconi anaemia (FA) were conditioned for HLA-identical sibling bone marrow transplant (BMT) with reduced dose of cyclophosphamide (Cy) without radiation or antithymocyte globulin (ATG). The total dose of Cy was 140 mg/kg (n = 2) or 120 mg/kg (n = 7). The median patient age was 8 years (range 4-19)....

journal_title：British journal of haematology

authors： Flowers ME,Zanis J,Pasquini R,Deeg HJ,Ribeiro R,Longton G,Medeiros CR,Doney K,Sanders J,Bryant J

更新日期：1996-03-01 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：:Approximately 31 patients with chronic myelogenous leukaemia (CML) are documented in the literature who survived more than 10 years after diagnosis. We present a CML-patient whose survival of 27 years is probably the longest reported so far. The analysis of the course of disease in these patients revealed that the dur...

journal_title：British journal of haematology

authors： Prischl FC,Haas OA,Lion T,Eyb R,Schwarzmeier JD

更新日期：1989-03-01 00:00:00