ELANE mutant-specific activation of different UPR pathways in congenital neutropenia.

abstract：:MPLW515L/K and JAK2V617F can co-exist in myelofibrosis with myeloid metaplasia (MMM). The chronology of clonal emergence was studied in three such cases using serially stored bone marrow. At diagnosis, a major MPL515 mutant clone was accompanied by a minor JAK2V617F clone in all three instances. At 25 time points over...

journal_title：British journal of haematology

authors： Lasho TL,Pardanani A,McClure RF,Mesa RA,Levine RL,Gilliland DG,Tefferi A

更新日期：2006-12-01 00:00:00

abstract：:Nd-HPFH are haematological conditions which are natural models to aid understanding of the haemoglobin (Hb) switch. In this paper we describe a new non-deletional hereditary persistence of fetal haemoglobin (nd-HPFH) associated with the highest Hb F level observed to date (up to 49% without haemopoietic stress). Seque...

journal_title：British journal of haematology

authors： Pissard S,M'rad A,Beuzard Y,Roméo PH

更新日期：1996-10-01 00:00:00

abstract：:A primary analysis of the ASPIRE study found that the addition of carfilzomib to lenalidomide and dexamethasone (carfilzomib group) significantly improved progression-free survival (PFS) compared with lenalidomide and dexamethasone alone (control group) in patients with relapsed multiple myeloma (RMM). This post hoc a...

journal_title：British journal of haematology

authors： Dimopoulos MA,Stewart AK,Masszi T,Špička I,Oriol A,Hájek R,Rosiñol L,Siegel D,Mihaylov GG,Goranova-Marinova V,Rajnics P,Suvorov A,Niesvizky R,Jakubowiak A,San-Miguel J,Ludwig H,Palumbo A,Obreja M,Aggarwal S,Moreau P

更新日期：2017-05-01 00:00:00

abstract：:In an ongoing effort to identify point mutations causing beta-thalassaemia, we have found two previously unreported mutations which are located in the Poly A site of the beta-globin gene. The screening programme used amplified DNA and dot-blot hybridization with several 32P-labelled oligonucleotide probes. DNA samples...

journal_title：British journal of haematology

authors： Jankovic L,Efremov GD,Petkov G,Kattamis C,George E,Yang KG,Stoming TA,Huisman TH

更新日期：1990-05-01 00:00:00

abstract：:We report a child with acute promyelocytic leukaemia (APL) who was treated with etoposide (VP16) for Langerhans cell histiocytosis (LCH). A 3-year-old Japanese girl was diagnosed as having LCH. She was treated with combination chemotherapy using VP16 and prednisolone. 56 months after beginning the chemotherapy she dev...

journal_title：British journal of haematology

authors： Matsuzaki A,Inamitsu T,Watanabe T,Ohga S,Ishii E,Nagotoshi Y,Tasaka H,Suda M,Ueda K

更新日期：1994-04-01 00:00:00

abstract：:It is widely recognized that thrombosis is the major event in the evolution of stable vascular disease to unstable ischaemic syndromes including myocardial infarction and stroke. The purpose of this case-control study was to establish clinical and laboratory data on the possible relationship between specific component...

journal_title：British journal of haematology

authors： Freeburn JC,Wallace JM,Strain JJ,Sinnamon DG,Craig BM,Johnson D,Gilmore WS

更新日期：1998-07-01 00:00:00

abstract：:The existence of functional gap junctions between haematopoietic progenitor cells (HPCs) and stromal cells of the haematopoietic microenvironment in the human system is a controversial issue. Primary CD34+ HPCs isolated from leukapheresis products were co-incubated with the human fibroblastoid bone marrow stromal cell...

journal_title：British journal of haematology

authors： Dürig J,Rosenthal C,Halfmeyer K,Wiemann M,Novotny J,Bingmann D,Dührsen U,Schirrmacher K

更新日期：2000-11-01 00:00:00

abstract：:Haemophilia A is an attractive target for gene therapy. We designed a haemophilia A gene therapy strategy involving the genetic modification of haematopoietic stem cells to achieve tissue-specific expression of a factor VIII (FVIII) transgene in the megakaryocytic lineage. Platelets would then serve as vehicles to sto...

journal_title：British journal of haematology

authors： Rodriguez MH,Plantier JL,Enjolras N,Réa M,Leboeuf M,Uzan G,Négrier C

更新日期：2004-12-01 00:00:00

abstract：:Marizomib, a natural marine product, is an irreversible proteasome inhibitor currently under investigation in relapsed-refractory multiple myeloma (RRMM) and malignant glioma. Central nervous system-multiple myeloma (CNS-MM) is a rare manifestation of extra-medullary disease with few therapeutic options, highlighting ...

journal_title：British journal of haematology

authors： Badros A,Singh Z,Dhakal B,Kwok Y,MacLaren A,Richardson P,Trikha M,Hari P

更新日期：2017-04-01 00:00:00

abstract：:Sepsis may be associated with activation of the coagulation system and, in its most severe form, may result in disseminated intravascular coagulation (DIC). Initially, there is thrombosis primarily affecting small and medium sized vessels and contributing to organ dysfunction, but continued activation results in consu...

journal_title：British journal of haematology

authors： Scully M,Levi M

更新日期：2019-04-01 00:00:00

abstract：:A stroma free platelet lysate supernatant (PLS) was prepared by repeatedly freezing and thawing a human platelet suspension separated from platelet rich plasma by gel filtration. The material was potently thrombogenic in a stasis model in rabbits, but only when combined with a purified preparation of factor Xa. The do...

journal_title：British journal of haematology

authors： Giles AR,Nesheim ME,Hoogendoorn H,Tracy PB,Mann KG

更新日期：1982-07-01 00:00:00

abstract：:A 69-year-old woman presented with splenic marginal zone lymphoma associated with acquired von Willebrand's disease (AVWD). Laboratory abnormalities included markedly decreased plasma levels of factor VIII coagulant (C) activity (VIII:C 28%), von Willebrand's factor (VWF) antigen (Ag) (vWF:Ag < 6%), and VWF ristocetin...

journal_title：British journal of haematology

authors： Tefferi A,Hanson CA,Kurtin PJ,Katzmann JA,Dalton RJ,Nichols WL

更新日期：1997-03-01 00:00:00

abstract：:The impact of age, ethnicity and socio-economic deprivation in the era of novel anti-myeloma agents is unclear. Using linked national data from New Zealand, we evaluated the incidence, prevalence and overall survival (OS) of individuals who were diagnosed with myeloma between 2004 and 2016. The crude incidence rate in...

journal_title：British journal of haematology

authors： Chan HSH,Milne RJ

更新日期：2020-03-01 00:00:00

abstract：:Sixty patients with von Willebrand's disease belonging to 34 unrelated families were classified into the various types of the disease by analysis of the multimer patterns of von Willebrand factor. Type I disease was observed in 62% of the families, a finding similar to the recently published British and Swedish series...

journal_title：British journal of haematology

authors： Berliner SA,Seligsohn U,Zivelin A,Zwang E,Sofferman G

更新日期：1986-03-01 00:00:00

abstract：:Peripheral blood mononuclear cells (PBMNC) from 23 healthy subjects and 39 patients with B-cell chronic leukaemia (B-CLL) were assayed for ribonuclease H activity using as substrate the filter-immobilized synthetic homopolymer hybrid 3H-poly(rA):poly(dT). In 69% of the leukaemia patients examined enzyme activities wer...

journal_title：British journal of haematology

authors： Papaphilis AD,Kamper EF,Kattamis C,Pangalis GA

更新日期：1988-11-01 00:00:00

abstract：:Thrombocytopenia is common (40-65%) and potentially serious in myelodysplastic syndromes (MDS). A systematic review was conducted to determine the safety and efficacy of adding a thrombopoietin-receptor (THPO-R) agonist to standard MDS treatment. MEDLINE, EMBASE and CENTRAL databases were searched. We included randomi...

journal_title：British journal of haematology

authors： Prica A,Sholzberg M,Buckstein R

更新日期：2014-12-01 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：:We explored the prognostic factors for children with very high-risk (VHR) Philadelphia chromosome (Ph) negative B-cell acute lymphoblastic leukaemia (B-ALL) and compared the therapeutic effects of intensive chemotherapy and unmanipulated haploidentical haematopoietic stem cell transplantation (haplo-HSCT) as post-remi...

journal_title：British journal of haematology

authors： Xue YJ,Suo P,Huang XJ,Lu AD,Wang Y,Zuo YX,Yan CH,Wu J,Kong J,Zhang XH,Chen YH,Jia YP,Liu KY,Han W,Xu LP,Zhang LP,Cheng YF

更新日期：2020-03-01 00:00:00

abstract：:We describe a BCR/ABL rearrangement positive but Philadelphia chromosome negative status in a 9-year-old boy suffering from an acute myelogenous leukaemia (AML). This case was detected in a prospective PCR screening procedure including 21 children with newly diagnosed AML and 150 children with acute lymphoblastic leuk...

journal_title：British journal of haematology

authors： Borkhardt A,Repp R,Harbott J,Lakomek M,Janssen JW,Schlieben S,Bartram CR,Lampert F

更新日期：1993-06-01 00:00:00

abstract：:Thrombomodulin (TM) is an endothelial cell glycoprotein that acts as an anticoagulant. Mutation in the TM gene is a potential risk factor for thrombosis. The first TM mutation identified was a heterozygous substitution of T for G at nucleotide position 1456, which predicted Asp468 with Tyr in a Ser/Thr-rich domain. To...

journal_title：British journal of haematology

authors： Nakazawa F,Koyama T,Saito T,Shibakura M,Yoshinaga H,Chung DH,Kamiyama R,Hirosawa S

更新日期：1999-08-01 00:00:00

abstract：:A double-blind, crossover, placebo-controlled study of the effect of vitamin E on platelet functions was performed on nine splenectomized and 16 non-splenectomized beta-thalassaemia/haemoglobin E (beta-thalassaemia/HbE) patients. The patients were supplemented with a daily dose of vitamin E (525 IU) for 3 months. The ...

journal_title：British journal of haematology

authors： Unchern S,Laoharuangpanya N,Phumala N,Sipankapracha P,Pootrakul P,Fucharoen S,Wanachivanawin W,Chantharaksri U

更新日期：2003-11-01 00:00:00

abstract：:Measurements were made of levels of D-dimer in plasma and serum, thrombin-antithrombin complex (TAT) in plasma and fibrinogen/fibrin fragment E antigen (FgE) in serum in a normal healthy control group and in patients with a range of disorders associated with hypercoagulability. Levels were determined in 31 normal heal...

journal_title：British journal of haematology

authors： Boisclair MD,Lane DA,Wilde JT,Ireland H,Preston FE,Ofosu FA

更新日期：1990-04-01 00:00:00

abstract：:Proteasome inhibition induces the accumulation of aggregated misfolded/ubiquitinated proteins in the aggresome; conversely, histone deacetylase 6 (HDAC6) inhibition blocks aggresome formation. Although this rationale has been the basis of proteasome inhibitor (PI) and HDAC6 inhibitor combination studies, the role of d...

journal_title：British journal of haematology

authors： Mishima Y,Santo L,Eda H,Cirstea D,Nemani N,Yee AJ,O'Donnell E,Selig MK,Quayle SN,Arastu-Kapur S,Kirk C,Boise LH,Jones SS,Raje N

更新日期：2015-05-01 00:00:00

abstract：:Patients with sickle cell disease (SCD) with high fetal haemoglobin (HbF) tend to have a lower incidence of complications and longer survival due to inhibition of deoxyhaemoglobin S (HbS) polymerisation by HbF. HbF-containing cells, namely F cells, are strongly influenced by genetic factors. We measured the percentage...

journal_title：British journal of haematology

authors： Urio F,Nkya S,Rooks H,Mgaya JA,Masamu U,Zozimus Sangeda R,Mmbando BP,Brumat M,Mselle T,Menzel S,Luzzatto L,Makani J

更新日期：2020-10-19 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：:The incidence, characteristics and risk factors for the development of osteonecrosis of the jaw (ONJ) were evaluated among 303 myeloma patients. Only patients who received bisphosphonates developed ONJ (28/254; 11%). Zoledronic acid produced 9.5-fold greater risk for developing ONJ than pamidronate alone (P = 0.042) a...

journal_title：British journal of haematology

authors： Zervas K,Verrou E,Teleioudis Z,Vahtsevanos K,Banti A,Mihou D,Krikelis D,Terpos E

更新日期：2006-09-01 00:00:00

abstract：:We studied 13 patients with rheumatoid arthritis (RA) and gold-induced thrombocytopenia. Platelet-specific autoantibodies of the IgG and often also of the IgM class were detected by immunofluorescence on the patient's platelets and in ether eluates from these platelets. In nine patients we also detected autoantibodies...

journal_title：British journal of haematology

authors： von dem Borne AE,Pegels JG,van der Stadt RJ,van der Plas-van Dalen CM,Helmerhorst FM

更新日期：1986-07-01 00:00:00

abstract：:Osteonecrosis is a disabling complication in children and young adults with acute lymphoblastic leukaemia. It can affect any or multiple joints but the hip and knee are most frequently involved and a cause of long-term disability. The problem is almost exclusively that of older children and young adults of whom over 7...

journal_title：British journal of haematology

authors： Vora A

更新日期：2011-12-01 00:00:00

abstract：:Adult T-cell leukaemia lymphoma (ATLL) is an aggressive disease caused by the human T-lymphotropic virus 1 (HTLV-I) with a short survival. Responses to interferon alpha (IFN-alpha) and zidovudine (AZT) have been documented but not with long-term follow-up. We treated 15 ATLL patients with IFN and AZT. Eleven patients ...

journal_title：British journal of haematology

authors： Matutes E,Taylor GP,Cavenagh J,Pagliuca A,Bareford D,Domingo A,Hamblin M,Kelsey S,Mir N,Reilly JT

更新日期：2001-06-01 00:00:00

abstract：:Human megakaryocytes have been isolated from marrow obtained from ribs removed at thoracotomy. All but one of the patients had normal pre-operative platelet and leucocyte counts. Megakaryocytes averaged 0.37% of all cells in marrow cell suspensions from nine consecutive subjects. A 283-fold purification (to 10.3%) was...

journal_title：British journal of haematology

authors： Levine RF

更新日期：1980-07-01 00:00:00