当前位置： SCI文献检索 > BRITISH JOURNAL OF HAEMATOLOGY期刊下所有文献 > Advances in understanding of pathogenesis of aHUS and HELLP.

Advances in understanding of pathogenesis of aHUS and HELLP.

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 of function of the alternative pathway, due to mutations in complement regulatory proteins and activating components. Recently, the same complement mutation identified in multiple patients with aHUS was found in a patient with the HELLP syndrome. The pathogeneses of both diseases are reviewed focusing on the role of the complement system and how its dysfunction could result in a thrombotic microangiopathy in the kidney in the case of aHUS and in the liver in the case of the HELLP syndrome.

journal_name

Br J Haematol

journal_title

British journal of haematology

authors

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

doi

10.1111/j.1365-2141.2008.07324.x

subject

Has Abstract

pub_date

2008-11-01 00:00:00

pages

336-48

issue

3

eissn

0007-1048

issn

1365-2141

pii

BJH7324

journal_volume

143

pub_type

杂志文章,评审

相关文献

BRITISH JOURNAL OF HAEMATOLOGY文献大全
  • A simplified statistical method for local INR using linear regression. European Concerted Action on Anticoagulation.

    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

    pub_type： 杂志文章,多中心研究

    doi：10.1046/j.1365-2141.1997.d01-3498.x

    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

  • Bone mineralization and turnover in children with congenital neutropenia, and its relationship to treatment with recombinant human granulocyte-colony stimulating factor.

    abstract：:Bone mineral content (BMC) of the radius was measured using single photon absorptiometry (SPA) in nine children with congenital neutropenia. Five had normal values. Two children with severe congenital neutropenia (SCN) had low BMC, and two boys with Schwachman syndrome had biochemistry suggestive of rickets. ...

    journal_title：British journal of haematology

    pub_type： 杂志文章,多中心研究

    doi：10.1046/j.1365-2141.1997.1302945.x

    authors： Fewtrell MS,Kinsey SE,Williams DM,Bishop NJ

    更新日期：1997-06-01 00:00:00

  • Comparative analyses of megakaryocytes derived from cord blood and bone marrow.

    abstract：:Thrombocytopenia is typically observed in patients undergoing cord blood transplantation. We hypothesized that delayed recovery of the platelet count might be caused by defects in the megakaryocytic differentiation pathway of cord blood progenitors. To test this hypothesis, we compared the features of in vitro megakar...

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1046/j.1365-2141.2000.01854.x

    authors： Miyazaki R,Ogata H,Iguchi T,Sogo S,Kushida T,Ito T,Inaba M,Ikehara S,Kobayashi Y

    更新日期：2000-03-01 00:00:00

  • Multilineage involvement in hypereosinophilic syndrome terminating in granulocytic sarcoma and leukaemic transformation with trisomy 8.

    abstract：:We report a patient with hypereosinophilic syndrome (HES), which, 8 years later, transformed into granulocytic sarcoma in the brain and, subsequently, into acute myelocytic leukaemia. Repeated chromosome analyses showed a normal karyotype, until the time of leukaemic transformation when trisomy 8 was confirmed in cell...

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1046/j.1365-2141.2002.03922.x

    authors： Fujii N,Ikeda K,Takahashi N,Kojima K,Kobayashi Y,Ashiba A,Takenaka K,Fukuda S,Shinagawa K,Ishimaru F,Niiya K,Miura I,Tanimoto M,Harada M

    更新日期：2002-12-01 00:00:00

  • Detection of monoclonal plasma cells in the peripheral blood of patients with primary amyloidosis.

    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

    pub_type： 杂志文章

    doi：10.1046/j.1365-2141.1998.00583.x

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

    更新日期：1998-02-01 00:00:00

  • Unbalanced X-chromosome inactivation in haemopoietic cells from normal women.

    abstract：:We studied X-chromosome inactivation patterns in blood cells from normal females in three age groups: neonates (umbilical cord blood), 25-32 years old (young women group) and >75 years old (elderly women). Using PCR, the differential allele methylation status was evaluated on active and inactive X chromosomes at the h...

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1046/j.1365-2141.1998.00867.x

    authors： Tonon L,Bergamaschi G,Dellavecchia C,Rosti V,Lucotti C,Malabarba L,Novella A,Vercesi E,Frassoni F,Cazzola M

    更新日期：1998-09-01 00:00:00

  • Combined somatic mutation and copy number analysis in the survival of familial CLL.

    abstract：:Recurrent large-scale somatic copy number alterations (SCNAs), and somatic point mutations can be analysed to stratify patients with chronic lymphocytic leukaemia (CLL) into distinct prognostic groups. To investigate the relationship between SCNAs and somatic mutations, we performed whole-exome sequencing and single n...

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1111/bjh.15239

    authors： Zhou W,Goldin L,Wang M,McMaster ML,Jones K,Burdett L,Chanock SJ,Yeager M,Dean M,Caporaso NE

    更新日期：2018-06-01 00:00:00

  • Surface immunoglobulin on B lymphocytes as a potential target for specific peptide ligands in chronic lymphocytic leukaemia.

    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

    pub_type： 杂志文章

    doi：10.1046/j.0007-1048.2001.03311.x

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

    更新日期：2002-03-01 00:00:00

  • Myelofibrosis biology and contemporary management.

    abstract：:Myelofibrosis is an enigmatic myeloproliferative neoplasm, despite noteworthy strides in understanding its genetic underpinnings. Driver mutations involving JAK2, CALR or MPL in 90% of patients mediate constitutive JAK-STAT signaling which, in concert with epigenetic alterations (ASXL1, DNMT3A, SRSF2, EZH2, IDH1/2 mut...

    journal_title：British journal of haematology

    pub_type： 杂志文章,评审

    doi：10.1111/bjh.16576

    authors： Gangat N,Tefferi A

    更新日期：2020-10-01 00:00:00

  • Potential role of insulin-like growth factor binding protein-4 in the uncoupling of bone turnover in multiple myeloma.

    abstract：:Decreased bone formation plays an important role in the development of lytic lesions during the late stage of multiple myeloma (MM). Release of insulin-like growth factor binding protein-4 (IGFBP4) by tumour cells adjacent to bone may inhibit IGF-I-stimulated osteoblast growth and contribute to decreased bone formatio...

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1046/j.1365-2141.1999.01243.x

    authors： Feliers D,Woodruff K,Abboud S

    更新日期：1999-03-01 00:00:00

  • A 15q24 microdeletion in transient myeloproliferative disease (TMD) and acute megakaryoblastic leukaemia (AMKL) implicates PML and SUMO3 in the leukaemogenesis of TMD/AMKL.

    abstract：:Transient myeloproliferative disorder (TMD) of the newborn and acute megakaryoblastic leukaemia (AMKL) in children with Down syndrome (DS) represent paradigmatic models of leukaemogenesis. Chromosome 21 gene dosage effects and truncating mutations of the X-chromosomal transcription factor GATA1 synergize to trigger TM...

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1111/j.1365-2141.2012.09028.x

    authors： Haemmerling S,Behnisch W,Doerks T,Korbel JO,Bork P,Moog U,Hentze S,Grasshoff U,Bonin M,Rieß O,Janssen JW,Jauch A,Bartram CR,Reinhardt D,Koch KA,Bandapalli OR,Kulozik AE

    更新日期：2012-04-01 00:00:00

  • Multidrug resistance mechanisms in chronic lymphocytic leukaemia.

    abstract：:We evaluated the presence of P-glycoprotein (P-gp)-170, multidrug resistance protein (MRP), lung resistance protein (LRP)-56 and Bcl-2 in CD19-positive cells from 100 cases of chronic lymphocytic leukaemia (CLL). P-gp-170 was found in 73% of the CLL cases with no significant difference regarding stage or previous trea...

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1046/j.0007-1048.2002.03344.x

    authors： Consoli U,Santonocito A,Stagno F,Fiumara P,Privitera A,Parisi G,Giustolisi GM,Pavone B,Palumbo GA,Di Raimondo F,Milone G,Guglielmo P,Giustolisi R

    更新日期：2002-03-01 00:00:00

  • The use of direct oral anticoagulants in chronic kidney disease.

    abstract：:Increasing use of direct oral anticoagulants (DOACs) has made management of non-valvular atrial fibrillation and venous thromboembolism easier in most patients. But the presence of co-existing renal impairment could render the use of DOACs problematic because all of these drugs have varying degrees of renal excretion....

    journal_title：British journal of haematology

    pub_type： 杂志文章,评审

    doi：10.1111/bjh.15564

    authors： Parker K,Thachil J

    更新日期：2018-10-01 00:00:00

  • Variable differentiation patterns of acute myelogenous leukaemia blasts in liquid suspension cultures.

    abstract：:To study the ability of acute myelogenous leukaemia blasts to spontaneously differentiate in vitro, bone marrow and/or blood mononuclear cells from 63 patients with acute myelogenous leukaemia were incubated in liquid suspension cultures containing human serum, without addition of chemical inducers of differentiation....

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1111/j.1365-2141.1987.tb01296.x

    authors： Baer MR,Dessypris EN,Stein RS,Cousar JB,Krantz SB

    更新日期：1987-06-01 00:00:00

  • 'Lymphoid' blast crisis of chronic myeloid leukaemia is associated with distinct clinicohaematological features.

    abstract：:It has been suggested that in blast crisis (BC) of chronic myeloid leukaemia (CML) the clinical and laboratory features of patients with 'lymphoid' phenotype differ from those of patients with non-lymphoid BC. In order to assess any differences, 97 patients consecutively diagnosed with BC that followed a known chronic...

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1046/j.1365-2141.1998.00542.x

    authors： Cervantes F,Villamor N,Esteve J,Montoto S,Rives S,Rozman C,Montserrat E

    更新日期：1998-01-01 00:00:00

  • Interleukin-6 inhibits the chemotaxis of human malignant plasma cell lines.

    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

    pub_type： 杂志文章

    doi：10.1046/j.1365-2141.1996.d01-1691.x

    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

  • Relapse of aplastic anaemia after immunosuppressive treatment: a report from the European Bone Marrow Transplantation Group SAA Working Party.

    abstract：:This study was designed to determine the incidence of relapse and factors predictive for relapse in 719 patients with severe aplastic anaemia (SAA) after immunosuppressive treatment (IS). Patients developing myelodysplasia or acute leukaemia after IS, and patients receiving a transplant, were excluded from this analys...

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1111/j.1365-2141.1993.tb03181.x

    authors： Schrezenmeier H,Marin P,Raghavachar A,McCann S,Hows J,Gluckman E,Nissen C,van't Veer-Korthof ET,Ljungman P,Hinterberger W

    更新日期：1993-10-01 00:00:00

  • Locus assignment of human alpha globin mutations by selective amplification and direct sequencing.

    abstract：:We describe a simple approach for molecular characterization and locus assignment of structural mutants by direct sequencing of enzymatically amplified DNA selective to alpha 1 and alpha 2 globin gene regions. Nucleotide substitution of two structural variants (Stanleyville II alpha 2(78Lys) and J Mexico alpha 2(54Glu...

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1111/j.1365-2141.1990.tb07884.x

    authors： Dodé C,Rochette J,Krishnamoorthy R

    更新日期：1990-10-01 00:00:00

  • Humanized CD52 monoclonal antibody Campath-1H as first-line treatment in chronic lymphocytic leukaemia.

    abstract：:The humanized CD52 monoclonal antibody Campath-1H was used as first-line therapy in nine patients with progressive chronic lymphocytic leukaemia (CLL). Intravenous (n = 5) or subcutaneous (n = 4) injections (up to 30 mg/inj.) were given three times a week for a maximum of 18 weeks. Three patients achieved a complete r...

    journal_title：British journal of haematology

    pub_type： 临床试验,杂志文章

    doi：10.1046/j.1365-2141.1996.450989.x

    authors： Osterborg A,Fassas AS,Anagnostopoulos A,Dyer MJ,Catovsky D,Mellstedt H

    更新日期：1996-04-01 00:00:00

  • Recombinant human erythropoietin is effective in correcting erythropoietin-deficient anaemia after allogeneic bone marrow transplantation.

    abstract：:Two children affected by severe aplastic anaemia (SAA) underwent allogeneic bone marrow transplantation (BMT) using partially matched family donors. In both cases there was a successful engraftment of donor haemopoietic stem cells. However, after an initial erythropoietic recovery, 5 months following BMT both children...

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1111/j.1365-2141.1992.tb04571.x

    authors： Locatelli F,Pedrazzoli P,Barosi G,Zecca M,Porta F,Liberato L,Gambarana D,Nespoli L,Cazzola M

    更新日期：1992-04-01 00:00:00

  • Amplified segment in the 'Down syndrome critical region' on HSA21 shared between Down syndrome and euploid AML-M0 excludes RUNX1, ERG and ETS2.

    abstract：:Children with Down syndrome have a 20- to 50-fold increased risk of acute lymphocytic or myeloid leukaemia. Whole or partial gains of chromosome 21 have been described in multiple childhood leukaemias, and have recently been reported as a likely primary event in B-precursor-acute lymphoblastic leukaemia. It is unclear...

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1111/j.1365-2141.2011.08985.x

    authors： Canzonetta C,Hoischen A,Giarin E,Basso G,Veltman JA,Nacheva E,Nizetic D,Groet J

    更新日期：2012-04-01 00:00:00

  • Antenatal screening for fetal alloimmune thrombocytopenia: the results of a pilot study.

    abstract：:Feto-maternal incompatibility for the human platelet antigen HPA-1a is an important cause of severe fetal thrombocytopenia. The incidence is 1 in 1000-2000 pregnancies, which is more common than other conditions for which screening is presently carried out. Antenatal diagnosis and management are now available, but onl...

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1111/j.1365-2141.1995.tb05152.x

    authors： Doughty HA,Murphy MF,Metcalfe P,Waters AH

    更新日期：1995-06-01 00:00:00

  • The risk of recurrent venous thromboembolism in pregnancy and puerperium without antithrombotic prophylaxis.

    abstract：:Whether or not pregnant women with a previous episode of venous thromboembolism (VTE) should receive antithrombotic prophylaxis is a matter of debate. In order to estimate the rate of recurrent deep venous thrombosis (DVT) or pulmonary embolism (PE) during pregnancy and puerperium we retrospectively investigated a coh...

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1111/j.1365-2141.2006.06317.x

    authors： De Stefano V,Martinelli I,Rossi E,Battaglioli T,Za T,Mannuccio Mannucci P,Leone G

    更新日期：2006-11-01 00:00:00

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

    abstract：:A number of studies have demonstrated induction of the unfolded protein response (UPR) in patients with severe congenital neutropenia (CN) harbouring mutations of ELANE, encoding neutrophil elastase. Why UPR is not activated in patients with cyclic neutropenia (CyN) carrying the same ELANE mutations is unclear. We eva...

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1111/bjh.13823

    authors： Nustede R,Klimiankou M,Klimenkova O,Kuznetsova I,Zeidler C,Welte K,Skokowa J

    更新日期：2016-01-01 00:00:00

  • Mutations in the erythrocyte chemokine receptor (Duffy) gene: the molecular basis of the Fya/Fyb antigens and identification of a deletion in the Duffy gene of an apparently healthy individual with the Fy(a-b-) phenotype.

    abstract：:The erythrocyte chemokine receptor, a receptor for Plasmodium vivax, carries the antigens of the Duffy blood group system. Sequence analysis of reticulocyte RNA from individuals of known Duffy phenotype showed that the Fya antigen differs from the Fyb antigen as a result of a single nucleotide difference (A131 or G) e...

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1111/j.1365-2141.1995.tb05202.x

    authors： Mallinson G,Soo KS,Schall TJ,Pisacka M,Anstee DJ

    更新日期：1995-08-01 00:00:00

  • The incidence of trisomy 3 in splenic lymphoma with villous lymphocytes: a study by FISH.

    abstract：:Splenic lymphoma with villous lymphocytes (SLVL) is a low-grade B-cell lymphoproliferative disorder characterized by splenomegaly and circulating villous lymphocytes. The relationship between SLVL and splenic marginal zone lymphoma (SMZL), a disorder with identical splenic histology to SLVL, is not clear. Previous stu...

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1046/j.1365-2141.1999.01209.x

    authors： Gruszka-Westwood AM,Matutes E,Coignet LJ,Wotherspoon A,Catovsky D

    更新日期：1999-03-01 00:00:00

  • Removal of cytomegalovirus DNA from donor blood by filtration.

    abstract：:Blood from five donors, previously shown to be positive for cytomegalovirus (CMV) DNA following polymerase chain reaction (PCR) amplification, was filtered through commercially available leucocyte filters. Analysis of pre- and post-filtration samples by PCR with ethidium bromide staining has shown that filtration was ...

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1111/j.1365-2141.1993.tb04703.x

    authors： Smith KL,Cobain T,Dunstan RA

    更新日期：1993-04-01 00:00:00

  • Tissue factor activity in human monocytes is regulated by plasma: implications for the high and low responder phenomenon.

    abstract：:The 'high and low responder phenomenon' of monocyte tissue factor (MTF) activity has been attributed to effects on monocytes by granulocytes, platelets and lipopolysaccharide (LPS). To study the possible contribution of plasma to the high and low responder phenomenon, we measured the MTF activity in isolated cryoprese...

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1046/j.1365-2141.2001.02545.x

    authors： Nijziel M,van Oerle R,van 't Veer C,van Pampus E,Lindhout T,Hamulyák K

    更新日期：2001-01-01 00:00:00

  • A five-year follow-up of untreated patients with chronic lymphocytic leukaemia treated with ofatumumab and chlorambucil: final analysis of the Complement 1 phase 3 trial.

    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

    pub_type： 杂志文章

    doi：10.1111/bjh.16625

    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

  • Endogenous lymphokine activated killer cell activity and cytogenetic response in chronic myelogenous leukaemia treated with alpha-interferon.

    abstract：:The capacity of alpha-interferon (alpha-IFN) to induce lymphokine activated killer (LAK) cytotoxicity in the absence of interleukin-2 (IL2) has prompted us to test whether or not its ability to reduce dramatically the number of Ph1+ clones in chronic myelogenous leukaemia (CML) patients is in part mediated through the...

    journal_title：British journal of haematology

    pub_type： 杂志文章

    doi：10.1111/j.1365-2141.1993.tb08275.x

    authors： Meseri A,Delwail V,Brizard A,Lecron JC,Pelletier D,Guilhot F,Tanzer J,Goube de Laforest P

    更新日期：1993-02-01 00:00:00