Complement in Thrombotic Microangiopathies: Unraveling Ariadne's Thread Into the Labyrinth of Complement Therapeutics

scientific article published on 27 February 2019

Complement in Thrombotic Microangiopathies: Unraveling Ariadne's Thread Into the Labyrinth of Complement Therapeutics is …
instance of (P31):
review articleQ7318358
scholarly articleQ13442814

External links are
P356DOI10.3389/FIMMU.2019.00337
P932PMC publication ID6413705
P698PubMed publication ID30891033

P50authorAchilles AnagnostopoulosQ42933010
P2093author name stringEleni Gavriilaki
Dimitrios C Mastellos
P2860cites workRecessive mutations in DGKE cause atypical hemolytic-uremic syndromeQ24337607
Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotypeQ24613907
Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpuraQ24614077
Alternative pathway of complement in children with diarrhea-associated hemolytic uremic syndromeQ24653523
Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcomeQ24685473
Eculizumab in severe Shiga-toxin-associated HUSQ28238525
Alternative pathway activation of complement by Shiga toxin promotes exuberant C3a formation that triggers microvascular thrombosisQ28239591
Shiga toxin activates complement and binds factor H: evidence for an active role of complement in hemolytic uremic syndromeQ28243693
Mutations in alternative pathway complement proteins in American patients with atypical hemolytic uremic syndromeQ28283865
A systematic review of eculizumab for atypical haemolytic uraemic syndrome (aHUS)Q28661698
Mutations in complement regulatory proteins predispose to preeclampsia: a genetic analysis of the PROMISSE cohortQ28741729
Complement: a key system for immune surveillance and homeostasisQ29615484
A murine model of HUS: Shiga toxin with lipopolysaccharide mimics the renal damage and physiologic response of human diseaseQ30442039
Genetic and intervention studies implicating complement C3 as a major target for the treatment of periodontitisQ30582298
Regulation of C3a receptor signaling in human mast cells by G protein coupled receptor kinasesQ31028372
The clinical features, risk factors and outcome of thrombotic thrombocytopenic purpura occurring after bone marrow transplantation.Q33337105
Upshaw-Schulman syndrome revisited: a concept of congenital thrombotic thrombocytopenic purpuraQ33338776
Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome following allogeneic HPC transplantation: a diagnostic dilemmaQ33359551
Predisposition to atypical hemolytic uremic syndrome involves the concurrence of different susceptibility alleles in the regulators of complement activation gene cluster in 1q32.Q33364613
The development of atypical haemolytic-uraemic syndrome is influenced by susceptibility factors in factor H and membrane cofactor protein: evidence from two independent cohortsQ33365530
Sirolimus and thrombotic microangiopathy after allogeneic hematopoietic stem cell transplantationQ33366979
Blood and marrow transplant clinical trials network toxicity committee consensus summary: thrombotic microangiopathy after hematopoietic stem cell transplantationQ33367451
von Willebrand factor-cleaving protease (ADAMTS13) in the course of stem cell transplantationQ33370723
Heme interacts with c1q and inhibits the classical complement pathwayQ34963775
Pre-eclampsia: pathophysiology, diagnosis, and managementQ35141777
Compstatin: a C3-targeted complement inhibitor reaching its prime for bedside interventionQ35236103
Clinical laboratory markers of inflammation as determinants of chronic graft-versus-host disease activity and NIH global severityQ35685660
Antigen-presenting cell-derived complement modulates graft-versus-host diseaseQ36005386
Reduced graft-versus-host disease in C3-deficient mice is associated with decreased donor Th1/Th17 differentiation.Q36219724
Clinical aspects and molecular basis of primary deficiencies of complement component C3 and its regulatory proteins factor I and factor H.Q36405585
Complement, a target for therapy in inflammatory and degenerative diseases.Q38614302
Heme: Modulator of Plasma Systems in Hemolytic DiseasesQ38732762
The role of CR3 (CD11b/CD18) and CR4 (CD11c/CD18) in complement-mediated phagocytosis and podosome formation by human phagocytes.Q38758239
Intermediate Follow-up of Pediatric Patients With Hemolytic Uremic Syndrome During the 2011 Outbreak Caused by E. coli O104:H4.Q38886279
ComplementopathiesQ39142842
Pathogenic Variants in Complement Genes and Risk of Atypical Hemolytic Uremic Syndrome Relapse after Eculizumab DiscontinuationQ39229604
Complement C3-Targeted Therapy: Replacing Long-Held Assertions with Evidence-Based Discovery.Q39246952
Coagulation induced by C3aR-dependent NETosis drives protumorigenic neutrophils during small intestinal tumorigenesis.Q39307644
Complement component C3 mediates Th1/Th17 polarization in human T-cell activation and cutaneous GVHD.Q39982138
Thrombotic Microangiopathy after Allogeneic Stem Cell Transplantation - a Comparison of Eculizumab Therapy and Conventional TherapyQ40071991
Airway smooth muscle cells enhance C3a-induced mast cell degranulation following cell-cell contact.Q40448379
Platelet-activating factor and kinin-dependent vascular leakage as a novel functional activity of the soluble terminal complement complexQ40489320
Eculizumab in Pregnant Patients with Paroxysmal Nocturnal HemoglobinuriaQ40559071
Typical Hus: Evidence of Acute Phase Complement Activation from a Daycare Outbreak.Q40652002
Terminal Complement Blockade after Hematopoietic Stem Cell Transplantation Is Safe without Meningococcal Vaccination.Q40722182
Loss of DGKε induces endothelial cell activation and death independently of complement activationQ41700443
Thrombotic microangiopathy in blood and marrow transplant patients receiving tacrolimus or cyclosporine A.Q44272977
Decreased cold-insoluble globulin in congenital thrombocytopenia (Upshaw-Schulman syndrome)Q44561984
Risk factors, management, and outcomes of hemolysis, elevated liver enzymes, and low platelets syndrome and elevated liver enzymes, low platelets syndromeQ44577112
Urinary excretion of C5b-9 in severe preeclampsia: tipping the balance of complement activation in pregnancyQ45804466
Genetic Susceptibility to Hemolytic-Uremic Syndrome after Shiga Toxin-Producing Escherichia Coli (STEC) Infection: a Centers for Disease Control and Prevention (CDC) FoodNet Study.Q46030044
Prospective study on the behaviour of the metalloprotease ADAMTS13 and of von Willebrand factor after bone marrow transplantationQ33371965
Diagnostic criteria for hematopoietic stem cell transplant-associated microangiopathy: results of a consensus process by an International Working GroupQ33373889
Spontaneous hemolytic uremic syndrome triggered by complement factor H lacking surface recognition domainsQ33375335
Renal thrombotic microangiopathy after hematopoietic cell transplant: role of GVHD in pathogenesisQ33382787
Posttransplant thrombotic microangiopathy: sensitivity of proposed new diagnostic criteriaQ33384578
Thrombomodulin mutations in atypical hemolytic-uremic syndromeQ33385474
Comparison of thrombotic microangiopathy after allogeneic hematopoietic cell transplantation with high-dose or nonmyeloablative conditioningQ33386036
Hemolytic uremic syndrome and death in persons with Escherichia coli O157:H7 infection, foodborne diseases active surveillance network sites, 2000-2006.Q33386636
DEAP-HUS: deficiency of CFHR plasma proteins and autoantibody-positive form of hemolytic uremic syndromeQ33388274
Genetics and complement in atypical HUS.Q33389945
Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study GroupQ33390678
Plasmatherapy in atypical hemolytic uremic syndromeQ33392018
Complement activation on platelet-leukocyte complexes and microparticles in enterohemorrhagic Escherichia coli-induced hemolytic uremic syndromeQ33394691
Small vessels, big trouble in the kidneys and beyond: hematopoietic stem cell transplantation-associated thrombotic microangiopathyQ33395413
Renal thrombotic microangiopathy associated with chronic graft-versus-host disease after allogeneic hematopoietic stem cell transplantation.Q33396969
Common genetic variants in complement genes other than CFH, CD46 and the CFHRs are not associated with aHUS.Q33398635
A prevalent C3 mutation in aHUS patients causes a direct C3 convertase gain of functionQ33399198
Need for long-term follow-up in enterohemorrhagic Escherichia coli-associated hemolytic uremic syndrome due to late-emerging sequelaeQ33400221
Validation of treatment strategies for enterohaemorrhagic Escherichia coli O104:H4 induced haemolytic uraemic syndrome: case-control studyQ33402456
Lack of the lectin-like domain of thrombomodulin worsens Shiga toxin-associated hemolytic uremic syndrome in miceQ33403090
Use of eculizumab for atypical haemolytic uraemic syndrome and C3 glomerulopathiesQ33403607
Thrombotic microangiopathy associated with sirolimus level after allogeneic hematopoietic cell transplantation with tacrolimus/sirolimus-based graft-versus-host disease prophylaxisQ33403897
Best supportive care and therapeutic plasma exchange with or without eculizumab in Shiga-toxin-producing E. coli O104:H4 induced haemolytic-uraemic syndrome: an analysis of the German STEC-HUS registryQ33404151
Atypical hemolytic uremic syndrome: what is it, how is it diagnosed, and how is it treated?Q33404805
Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adultsQ33405246
Combined complement gene mutations in atypical hemolytic uremic syndrome influence clinical phenotypeQ33406025
Complement activation by heme as a secondary hit for atypical hemolytic uremic syndromeQ33407898
Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndromeQ33408211
Atypical hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: clinically differentiating the thrombotic microangiopathiesQ33408216
Abnormalities in the alternative pathway of complement in children with hematopoietic stem cell transplant-associated thrombotic microangiopathyQ33408788
Diagnosis and management of complement mediated thrombotic microangiopathiesQ33413376
Complement factor B mutations in atypical hemolytic uremic syndrome-disease-relevant or benign?Q33414109
Discontinuation of eculizumab maintenance treatment for atypical hemolytic uremic syndrome: a report of 10 casesQ33414142
Biomarkers of terminal complement activation confirm the diagnosis of aHUS and differentiate aHUS from TTP.Q33414383
Genetics of atypical hemolytic uremic syndrome (aHUS).Q33415087
Diagnostic and risk criteria for HSCT-associated thrombotic microangiopathy: a study in children and young adultsQ33415603
Dynamics of complement activation in aHUS and how to monitor eculizumab therapyQ33416519
Syndromes of thrombotic microangiopathyQ33417044
Safe and effective use of eculizumab in the treatment of severe Shiga toxin Escherichia coli-associated hemolytic uremic syndromeQ33419554
Mapping interactions between complement C3 and regulators using mutations in atypical hemolytic uremic syndromeQ33419905
Use of Eculizumab in Patients With Allogeneic Stem Cell Transplant-Associated Thrombotic Microangiopathy: A Study From the SFGM-TC.Q33420170
Efficacy and safety of eculizumab in atypical hemolytic uremic syndrome from 2-year extensions of phase 2 studiesQ33420172
Eculizumab reduces complement activation, inflammation, endothelial damage, thrombosis, and renal injury markers in aHUS.Q33421669
Atypical aHUS: State of the art.Q33421720
An international consensus approach to the management of atypical hemolytic uremic syndrome in childrenQ33421861
Modified Ham test for atypical hemolytic uremic syndromeQ33421884
A national specialized service in England for atypical haemolytic uraemic syndrome-the first year's experienceQ33422224
Mechanism of Platelet Activation and Hypercoagulability by Antithymocyte Globulins (ATG).Q33422771
Thrombotic microangiopathy: focus on atypical hemolytic uremic syndrome.Q33423371
Variable Eculizumab Clearance Requires Pharmacodynamic Monitoring to Optimize Therapy for Thrombotic Microangiopathy after Hematopoietic Stem Cell Transplantation.Q33426602
Complement in hemolytic anemiaQ33427662
Disease Recurrence After Early Discontinuation of Eculizumab in a Patient With Atypical Hemolytic Uremic Syndrome With Complement C3 I1157T MutationQ33429735
Eculizumab is a safe and effective treatment in pediatric patients with atypical hemolytic uremic syndromeQ33430035
Direct evidence of complement activation in HELLP syndrome: A link to atypical hemolytic uremic syndromeQ33430320
Terminal Complement Inhibitor Eculizumab in Adult Patients With Atypical Hemolytic Uremic Syndrome: A Single-Arm, Open-Label TrialQ33431023
Defining the genetics of thrombotic microangiopathiesQ33432223
Small-molecule factor D inhibitors selectively block the alternative pathway of complement in paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndromeQ33436512
Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies.Q33436972
Syndrome of hemolysis, elevated liver enzymes, and low platelet count: a severe consequence of hypertension in pregnancyQ33484095
Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura.Q33499130
Acquired deficiency of von Willebrand factor-cleaving protease in a patient with thrombotic thrombocytopenic purpuraQ33502070
Comparative proteomic analysis of serum from patients with systemic sclerosis and sclerodermatous GVHD. Evidence of defective function of factor HQ33668640
Current evidence on the discontinuation of eculizumab in patients with atypical haemolytic uraemic syndromeQ33783126
The spectrum of severe preeclampsia: comparative analysis by HELLP (hemolysis, elevated liver enzyme levels, and low platelet count) syndrome classificationQ33864822
Immune cell-derived C3a and C5a costimulate human T cell alloimmunity.Q34370940
Clinical utility of strict diagnostic criteria for the HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome.Q34393266
Paroxysmal nocturnal hemoglobinuriaQ34431429
Understanding and managing HELLP syndrome: the integral role of aggressive glucocorticoids for mother and childQ34516456
Relapse of aHUS after discontinuation of therapy with eculizumab in a patient with aHUS and factor H mutationQ46159176
Eculizumab for the treatment of preeclampsia/HELLP syndromeQ46321630
Maternal-perinatal outcome associated with the syndrome of hemolysis, elevated liver enzymes, and low platelets in severe preeclampsia-eclampsiaQ46377642
The renaissance of complement therapeutics.Q47336749
Eculizumab treatment and impaired opsonophagocytic killing of meningococci by whole blood from immunized adultsQ48255838
Murine systemic thrombophilia and hemolytic uremic syndrome from a factor H point mutation.Q51213973
Contact activation of C3 enables tethering between activated platelets and polymorphonuclear leukocytes via CD11b/CD18.Q51741449
Eculizumab treatment in severe pediatric STEC-HUS: a multicenter retrospective study.Q51783073
Pregnancy-induced atypical haemolytic uremic syndrome: A new era with eculizumab.Q52595152
Statistical Validation of Rare Complement Variants Provides Insights into the Molecular Basis of Atypical Hemolytic Uremic Syndrome and C3 Glomerulopathy.Q52676485
Von Willebrand factor regulates complement on endothelial cells.Q52866918
Eculizumab therapy in adults with allogeneic hematopoietic cell transplant-associated thrombotic microangiopathy.Q52875784
A novel C5a receptor-tissue factor cross-talk in neutrophils links innate immunity to coagulation pathways.Q53598537
Transplant-associated thrombotic microangiopathy: an unresolved complication of unrelated allogeneic transplant for hematologic diseases.Q54306708
Germline mutations in the alternative pathway of complement predispose to HELLP syndrome.Q54955566
Genetic Analysis of 400 Patients Refines Understanding and Implicates a New Gene in Atypical Hemolytic Uremic SyndromeQ58095881
Eculizumab in atypical hemolytic uremic syndrome: strategies toward restrictive useQ58608395
Complement-mediated thrombotic microangiopathy associated with lupus nephritisQ58727886
Treatment of hemolytic uremic syndrome related to Bordetella pertussis infection -is plasma exchange or eculizumab use necessary?Q60044372
Heme Drives Susceptibility of Glomerular Endothelium to Complement Overactivation Due to Inefficient Upregulation of Heme Oxygenase-1Q60943310
Enhanced anaphylatoxin and terminal C5b-9 complement complex formation in patients with the syndrome of hemolysis, elevated liver enzymes, and low platelet countQ68565309
Complement c3a and c5a induce different signal transduction cascades in endothelial cellsQ74583305
Neutrophil adhesion to human endothelial cells is induced by the membrane attack complex: the roles of P-selectin and platelet activating factorQ77587533
Risk factors and severe outcome in thrombotic microangiopathy after allogeneic hematopoietic stem cell transplantationQ80252186
low body mass index is an independent risk factor for transplant-associated microangiopathy following total-body irradiation-based conditioning regimensQ81817471
Risk factor analysis for thrombotic microangiopathy after reduced-intensity or myeloablative allogeneic hematopoietic stem cell transplantationQ83931969
Eculizumab for the treatment of two recurrences of atypical hemolytic uremic syndrome in a kidney allograftQ84145395
Eculizumab cessation in atypical hemolytic uremic syndromeQ87968695
Brain microvascular endothelial cells exhibit lower activation of the alternative complement pathway than glomerular microvascular endothelial cellsQ88101871
C5b9 Formation on Endothelial Cells Reflects Complement Defects among Patients with Renal Thrombotic Microangiopathy and Severe HypertensionQ88954754
Safety and effectiveness of eculizumab for adult patients with atypical hemolytic-uremic syndrome in Japan: interim analysis of post-marketing surveillanceQ89357690
Hyponatremia: a new predictor of mortality in patients with Shiga toxin-producing Escherichia coli hemolytic uremic syndromeQ89366026
Haemolytic uraemic syndromeQ89373702
Expanding Complement Therapeutics for the Treatment of Paroxysmal Nocturnal HemoglobinuriaQ90390392
Safety and effectiveness of eculizumab for pediatric patients with atypical hemolytic-uremic syndrome in Japan: interim analysis of post-marketing surveillanceQ90435254
Hemolytic uremic syndrome associated with Bordetella pertussis infection in a 2-month-old infant carrying a pathogenic variant in complement factor HQ90602294
Transplant-associated thrombotic microangiopathy: Incidence, prognostic factors, morbidity, and mortality in allogeneic hematopoietic cell transplantationQ90771508
Emerging role of C5a/C5aR IL-17A axis in cGVHDQ90860842
Endothelium structure and function in kidney health and diseaseQ90871287
Safety profile after prolonged C3 inhibitionQ91456777
Blood clotting and traumatic injury with shock mediates complement-dependent neutrophil priming for extracellular ROS, ROS-dependent organ injury and coagulopathyQ91805694
P275copyright licenseCreative Commons Attribution 4.0 InternationalQ20007257
P407language of work or nameEnglishQ1860
P921main subjectHELLP syndromeQ1563513
thrombotic microangiopathyQ3312044
Complement inhibitorsQ56883896
disease susceptibilityQ65534164
body constitutionQ66771588
complement system proteinsQ67999591
P304page(s)337
P577publication date2019-02-27
P1433published inFrontiers in ImmunologyQ27723748
P1476titleComplement in Thrombotic Microangiopathies: Unraveling Ariadne's Thread Into the Labyrinth of Complement Therapeutics
P478volume10

Reverse relations

cites work (P2860)
Q96304836An Update in Drug-Induced Thrombotic Microangiopathy
Q92305390Atypical hemolytic uremic syndrome in a patient with protein-losing enteropathy

Search more.