Thrombotic Microangiopathies, New England Journal of Medicine, vol.347, issue.8, p.589600, 2002. ,
DOI : 10.1056/NEJMra020528
Improved survival in thrombotic thrombocytopenic purpurahemolytic uremic syndrome. Clinical experience in 108 patients, N Engl J Med. 8 août, vol.325, issue.6, p.398403, 1991. ,
Terminal Complement Inhibitor Eculizumab in Adult Patients With Atypical Hemolytic Uremic Syndrome: A Single-Arm, Open-Label Trial, American Journal of Kidney Diseases, vol.68, issue.1, p.8493, 2016. ,
DOI : 10.1053/j.ajkd.2015.12.034
Efficacy and safety of first-line rituximab in severe, acquired thrombotic thrombocytopenic purpura with a suboptimal response to plasma exchange. Experience of the French Thrombotic Microangiopathies Reference Center, Critical Care Medicine, vol.40, issue.1, p.10411, 2012. ,
DOI : 10.1097/CCM.0b013e31822e9d66
Caplacizumab for Acquired Thrombotic Thrombocytopenic Purpura, New England Journal of Medicine, vol.374, issue.6, p.51122, 2016. ,
DOI : 10.1056/NEJMoa1505533
Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies, Journal of Thrombosis and Haemostasis, vol.91, issue.Suppl. 1, p.31222, 2017. ,
DOI : 10.1097/MD.0b013e3182603598
Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy, The Lancet Haematology, vol.3, issue.5, pp.237-245, 2016. ,
DOI : 10.1016/S2352-3026(16)30018-7
Diagnostic and therapeutic challenges in the thrombotic thrombocytopenic purpura and hemolytic uremic syndromes, Hematol Am Soc Hematol Educ Program, vol.2012, p.6049, 2012. ,
What's new in the diagnosis and pathophysiology of thrombotic thrombocytopenic purpura, Hematology, vol.2015, issue.1, p.6316, 2015. ,
DOI : 10.1182/asheducation-2015.1.631
An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries; an undescribed disease, Am J Med. nov, vol.13, issue.5, p.5679, 1952. ,
Unusually Large Plasma Factor VIII: von Willebrand Factor Multimers in Chronic Relapsing Thrombotic Thrombocytopenic Purpura, New England Journal of Medicine, vol.307, issue.23, p.14325, 1982. ,
DOI : 10.1056/NEJM198212023072306
Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis, Blood. 15 mai, vol.87, issue.10, p.422334, 1996. ,
Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura, Blood. 1 mai, vol.89, issue.9, p.3097103, 1997. ,
Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion, Blood. 15 mai, vol.87, issue.10, p.423544, 1996. ,
Localization of ADAMTS13 to the stellate cells of human liver, Blood, vol.106, issue.3, p.9224, 2005. ,
DOI : 10.1182/blood-2005-01-0152
Platelet-derived VWF-cleaving metalloprotease ADAMTS-13, Journal of Thrombosis and Haemostasis, vol.62, issue.11, p.253644, 2005. ,
DOI : 10.1074/jbc.M301385200
Podocytes express ADAMTS13 in normal renal cortex and in patients with thrombotic thrombocytopenic purpura, British Journal of Haematology, vol.73, issue.5, p.65162, 2007. ,
DOI : 10.1038/labinvest.3700275
Mechanisms of microvascular thrombosis in thrombotic thrombocytopenic purpura, Kidney International, vol.75, issue.112, pp.11-14, 2009. ,
DOI : 10.1038/ki.2008.610
Immunohistochemistry of vascular lesion in thrombotic thrombocytopenic purpura, with special reference to factor VIII related antigen, Thrombosis Research, vol.38, issue.5, p.46979, 1985. ,
DOI : 10.1016/0049-3848(85)90180-X
The combined roles of ADAMTS13 and VWF in murine models of TTP, endotoxemia, and thrombosis, Blood, vol.111, issue.7, p.34527, 2008. ,
DOI : 10.1182/blood-2007-08-108571
Severe secondary deficiency of von Willebrand factor-cleaving protease (ADAMTS13) in patients with sepsis-induced disseminated intravascular coagulation: its correlation with development of renal failure, Blood, vol.107, issue.2, p.52834, 2006. ,
DOI : 10.1182/blood-2005-03-1087
Potential Role of ADAMTS13 in the Progression of Alcoholic Hepatitis, Current Drug Abuse Reviewse, vol.1, issue.2, p.18896, 2008. ,
DOI : 10.2174/1874473710801020188
von Willebrand factor-cleaving protease (ADAMTS13) activity in normal non-pregnant women, pregnant and postdelivery women, Thromb Haemost. déc, vol.92, issue.6, p.13206, 2004. ,
Mild to moderate reduction of a von Willebrand factor cleaving protease (ADAMTS-13) in pregnant women with HELLP microangiopathic syndrome, Haematologica. sept, vol.88, issue.9, p.102934, 2003. ,
ADAMTS13 mutations and polymorphisms in congenital thrombotic thrombocytopenic purpura, Hum Mutat. janv, vol.31, issue.1, p.119, 2010. ,
Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura, Nature, vol.228, issue.6855, p.48894, 2001. ,
DOI : 10.1126/science.3874428
Antibodies to von Willebrand Factor???Cleaving Protease in Acute Thrombotic Thrombocytopenic Purpura, New England Journal of Medicine, vol.339, issue.22, p.158594, 1998. ,
DOI : 10.1056/NEJM199811263392203
Acquired deficiency of von Willebrand factor-cleaving protease in a patient with thrombotic thrombocytopenic purpura, Blood. 15 avr, vol.91, issue.8, p.283946, 1998. ,
Pathogenicity of Anti-ADAMTS13 Autoantibodies in Acquired Thrombotic Thrombocytopenic Purpura, EBioMedicine, vol.2, issue.8, p.94252, 2015. ,
DOI : 10.1016/j.ebiom.2015.06.007
Persistence of circulating ADAMTS13-specific immune complexes in patients with acquired thrombotic thrombocytopenic purpura, Haematologica, vol.99, issue.4, p.77987, 2014. ,
DOI : 10.3324/haematol.2013.094151
Deficient activity of von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura in the setting of adult-onset Still's disease, Rheumatology, vol.45, issue.8, p.10467, 2006. ,
DOI : 10.1093/rheumatology/kel176
Occurrence of thrombotic thrombocytopenic purpura in a systemic lupus erythematosus patient with antiphospholipid antibodies in association with a decreased activity of von Willebrand factor-cleaving protease, Lupus, vol.11, issue.7, p.4634, 2002. ,
Thrombotic thrombocytopenic purpura as an initial presentation of primary Sjögren's syndrome, Clin Rheumatol. févr, vol.21, issue.1, p.579, 2002. ,
Human immunodeficiency virus associated thrombotic thrombocytopenic purpura - favourable outcome with plasma exchange and prompt initiation of highly active antiretroviral therapy, British Journal of Haematology, vol.142, issue.Suppl. 5, p.5159, 2011. ,
DOI : 10.1111/j.1365-2141.2008.07276.x
URL : https://hal.archives-ouvertes.fr/hal-00621304
Clinical importance of ADAMTS13 activity during remission in patients with acquired thrombotic thrombocytopenic purpura, Blood, vol.128, issue.17, p.21758, 2016. ,
DOI : 10.1182/blood-2016-06-724161
HLA-DRB1*11: a strong risk factor for acquired severe ADAMTS13 deficiency-related idiopathic thrombotic thrombocytopenic purpura in Caucasians, Journal of Thrombosis and Haemostasis, vol.76, issue.4, p.8569, 2010. ,
DOI : 10.1111/j.1538-7836.2010.03772.x
Human leukocyte antigen association in idiopathic thrombotic thrombocytopenic purpura: evidence for an immunogenetic link, Journal of Thrombosis and Haemostasis, vol.112, issue.2, p.25762, 2010. ,
DOI : 10.1111/j.1538-7836.2009.03692.x
Thrombotic Thrombocytopenic Purpura in Black People: Impact of Ethnicity on Survival and Genetic Risk Factors, PLOS ONE, vol.121, issue.7, p.156679, 2016. ,
DOI : 10.1371/journal.pone.0156679.s001
URL : https://hal.archives-ouvertes.fr/hal-01347684
High prevalence of infectious events in thrombotic thrombocytopenic purpura and genetic relationship with toll-like receptor 9 polymorphisms: experience of the French Thrombotic Microangiopathies Reference Center, Transfusion, vol.86, issue.2, p.38997, 2014. ,
DOI : 10.1002/ajh.22091
URL : https://hal.archives-ouvertes.fr/hal-01064343
Natural history of Upshaw-Schulman syndrome based on ADAMTS13 gene analysis in Japan, Journal of Thrombosis and Haemostasis, vol.92, issue.1, p.283301, 2011. ,
DOI : 10.1016/j.thromres.2006.07.012
Upshaw-Schulman Syndrome Revisited: A Concept of Congenital Thrombotic Thrombocytopenic Purpura, International Journal of Hematology, vol.88, issue.1, p.1018, 2001. ,
DOI : 10.1111/j.1442-200X.1995.tb03697.x
Incidence of obstetrical thrombotic thrombocytopenic purpura in a retrospective study within thrombocytopenic pregnant women. A difficult diagnosis and a treatable disease, BMC Pregnancy and Childbirth, vol.40, issue.1, p.137, 2015. ,
DOI : 10.1111/jog.12125
URL : https://hal.archives-ouvertes.fr/hal-01166878
Efficacy of a rituximab regimen based on B cell depletion in thrombotic thrombocytopenic purpura with suboptimal response to standard treatment: Results of a phase II, multicenter noncomparative study, American Journal of Hematology, vol.83, issue.12, pp.1246-51, 2016. ,
DOI : 10.1097/01.md.0000133622.03370.07
A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura, Blood, vol.118, issue.7, pp.1746-53, 2011. ,
DOI : 10.1182/blood-2011-03-341131
Twice-daily therapeutical plasma exchange-based salvage therapy in severe autoimmune thrombotic thrombocytopenic purpura: the French TMA Reference Center experience, European Journal of Haematology, vol.8, issue.2, pp.183-91, 2016. ,
DOI : 10.1111/j.1538-7836.2010.03818.x
Combination vincristine and plasma exchange as initial therapy in patients with thrombotic thrombocytopenic purpura: one institution's experience and review of the literature, Transfusion, vol.89, issue.1, pp.41-50, 2005. ,
DOI : 10.1182/blood-2002-05-1397
An evaluation of ciclosporin and corticosteroids individually as adjuncts to plasma exchange in the treatment of thrombotic thrombocytopenic purpura, British Journal of Haematology, vol.93, issue.1, pp.146-155, 2007. ,
DOI : 10.1055/s-2005-925476
Splenectomy for the treatment of thrombotic thrombocytopenic purpura, British Journal of Haematology, vol.1, issue.5, pp.768-76, 2005. ,
DOI : 10.1182/blood-2003-11-4035
Splenectomy and/or cyclophosphamide as salvage therapies in thrombotic thrombocytopenic purpura: the French TMA Reference Center experience, Transfusion, vol.48, issue.11, pp.2436-2480, 2012. ,
DOI : 10.1038/ki.2008.621
N-acetylcysteine reduces the size and activity of von Willebrand factor in human plasma and mice, Journal of Clinical Investigation, vol.121, issue.2, pp.593-603, 2011. ,
DOI : 10.1172/JCI41062DS1
-acetylcysteine: a case report, Transfusion, vol.37, issue.5, pp.1221-1225, 2014. ,
DOI : 10.1097/CCM.0b013e31819ffed4
ADAMTS13 Antibody Depletion by Bortezomib in Thrombotic Thrombocytopenic Purpura, New England Journal of Medicine, vol.368, issue.1, pp.90-92, 2013. ,
DOI : 10.1056/NEJMc1213206
Antibody Depletion by Bortezomib through Blocking of Antigen Presentation, New England Journal of Medicine, vol.368, issue.14, pp.1364-1369, 2013. ,
DOI : 10.1056/NEJMc1301264
Neutralization of inhibitory antibodies and restoration of therapeutic ADAMTS-13 activity levels in inhibitor-treated rats by the use of defined doses of recombinant ADAMTS-13, Journal of Thrombosis and Haemostasis, vol.67, issue.11, pp.2053-62, 2015. ,
DOI : 10.1097/MOH.0b013e328309ec62
Potential for Recombinant ADAMTS13 as an Effective Therapy for Acquired Thrombotic Thrombocytopenic PurpuraSignificance, Arteriosclerosis, Thrombosis, and Vascular Biology, vol.35, issue.11, pp.2336-2378, 2015. ,
DOI : 10.1161/ATVBAHA.115.306014
Gain-of-function ADAMTS13 variants that are resistant to autoantibodies against ADAMTS13 in patients with acquired thrombotic thrombocytopenic purpura. Blood, pp.3836-3879, 2012. ,
The in vitro effect of the new antithrombotic drug candidate ALX-0081 on blood samples of patients undergoing percutaneous coronary intervention, Thrombosis and Haemostasis, vol.106, issue.1, pp.165-71, 2011. ,
DOI : 10.1160/TH10-12-0804
Survival and relapse in patients with thrombotic thrombocytopenic purpura, Blood, vol.115, issue.8, pp.1500-1511, 2010. ,
DOI : 10.1182/blood-2009-09-243790
ADAMTS13 and anti-ADAMTS13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission, Haematologica, vol.93, issue.2, pp.232-241, 2008. ,
DOI : 10.3324/haematol.11739
Preemptive rituximab infusions after remission efficiently prevent relapses in acquired thrombotic thrombocytopenic purpura, Blood, vol.124, issue.2, pp.204-214, 2014. ,
DOI : 10.1182/blood-2014-01-550244
Development and validation of a predictive model for death in acquired severe ADAMTS13 deficiency-associated idiopathic thrombotic thrombocytopenic purpura: the French TMA Reference Center experience, Haematologica, vol.97, issue.8, pp.1181-1187, 2012. ,
DOI : 10.3324/haematol.2011.049676
URL : https://hal.archives-ouvertes.fr/inserm-00700485
Prognostic value of inhibitory anti-ADAMTS13 antibodies in adult-acquired thrombotic thrombocytopenic purpura, British Journal of Haematology, vol.138, issue.1, pp.66-74, 2006. ,
DOI : 10.1182/blood-2003-11-4035
URL : https://hal.archives-ouvertes.fr/hal-00453285
Multiple major morbidities and increased mortality during long-term follow-up after recovery from thrombotic thrombocytopenic purpura. Blood, pp.2023-2029, 2013. ,
Risk Factors for Autoimmune Diseases Development After Thrombotic Thrombocytopenic Purpura, Medicine, vol.94, issue.42, p.1598, 2015. ,
DOI : 10.1097/MD.0000000000001598
Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura, Kidney International, vol.60, issue.3, pp.831-877, 2001. ,
DOI : 10.1046/j.1523-1755.2001.060003831.x
Syndrome of hemolysis, thrombopenia and nephropathy in infancy, Pediatrics. févr, vol.29, pp.209-232, 1962. ,
HEMORRHAGIC COLITIS ASSOCIATED WITH A RARE ESCHERICHIA COLI SEROTYPE., The Pediatric Infectious Disease Journal, vol.2, issue.4, pp.681-686, 1983. ,
DOI : 10.1097/00006454-198307000-00031
SPORADIC CASES OF HAEMOLYTIC-URAEMIC SYNDROME ASSOCIATED WITH FAECAL CYTOTOXIN AND CYTOTOXIN-PRODUCING ESCHERICHIA COLI IN STOOLS, The Lancet, vol.321, issue.8325, pp.619-639, 1983. ,
DOI : 10.1016/S0140-6736(83)91795-6
Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome, The Lancet, vol.365, issue.9464, pp.1073-86, 2005. ,
DOI : 10.1016/S0140-6736(05)71144-2
A German outbreak of haemolytic uraemic syndrome, The Lancet, vol.378, issue.9796, pp.1057-1065, 2011. ,
DOI : 10.1016/S0140-6736(11)61217-8
Outbreak of Escherichia coli O104:H4 haemolytic uraemic syndrome in France: outcome with eculizumab, Nephrology Dialysis Transplantation, vol.29, issue.3, pp.565-72, 2014. ,
DOI : 10.1093/ndt/gft470
Non-enteropathic hemolytic uremic syndrome: causes and short-term course, American Journal of Kidney Diseases, vol.43, issue.6, pp.976-82, 2004. ,
DOI : 10.1053/j.ajkd.2004.02.010
Emerging and re-emerging diseases--epidemic enterohaemorrhagic infections 100 years after Shiga. South Afr Med J Suid-Afr Tydskr Vir Geneeskd, juill, vol.89, issue.7, pp.750-752, 1999. ,
Household transmission of haemolytic uraemic syndrome associated with Escherichia coli O104:H4, south-western France, Euro Surveill Bull Eur Sur Mal Transm Eur Commun Dis Bull. 4 août, vol.16, issue.31, 2011. ,
URL : https://hal.archives-ouvertes.fr/pasteur-01121005
The Induction of Apoptosis by Shiga Toxins and Ricin, Curr Top Microbiol Immunol, vol.357, pp.137-78, 2012. ,
DOI : 10.1007/82_2011_155
Enterohemorrhagic Escherichia coli Pathogenesis and the Host Response, Microbiology Spectrum, vol.2, issue.5, 2014. ,
DOI : 10.1128/microbiolspec.EHEC-0009-2013
A Novel Mechanism of Bacterial Toxin Transfer within Host Blood Cell-Derived Microvesicles, PLOS Pathogens, vol.12, issue.2, p.1004619, 2015. ,
DOI : 10.1371/journal.ppat.1004619.s004
Shiga toxin B subunits induce VWF secretion by human endothelial cells and thrombotic microangiopathy in ADAMTS13-deficient mice, Blood, vol.116, issue.18, pp.3653-3662, 2010. ,
DOI : 10.1182/blood-2010-02-271957
Hemolytic Uremic Syndrome, Journal of the American Society of Nephrology, vol.16, issue.4, pp.1035-50, 2005. ,
DOI : 10.1681/ASN.2004100861
O157:H7 Infections, New England Journal of Medicine, vol.342, issue.26, pp.1930-1936, 2000. ,
DOI : 10.1056/NEJM200006293422601
Association Between Azithromycin Therapy and Duration of Bacterial Shedding Among Patients With Shiga Toxin???Producing Enteroaggregative Escherichia coli O104:H4, JAMA, vol.307, issue.10, pp.1046-52, 2012. ,
DOI : 10.1001/jama.2012.264
Sporadic bloody diarrhoea-associated thrombotic thrombocytopenic purpura-haemolytic uraemic syndrome: an adult and paediatric comparison, British Journal of Haematology, vol.71, issue.5, pp.696-707, 2008. ,
DOI : 10.1056/NEJM200006293422601
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 registry, Nephrology Dialysis Transplantation, vol.27, issue.10, pp.3807-3822, 2012. ,
DOI : 10.1093/ndt/gfs394
Treatment of severe neurological deficits with IgG depletion through immunoadsorption in patients with Escherichia coli O104:H4-associated haemolytic uraemic syndrome: a prospective trial, The Lancet, vol.378, issue.9797, pp.1166-73, 2011. ,
DOI : 10.1016/S0140-6736(11)61253-1
Immunoadsorption in patients with haemolytic uraemic syndrome, The Lancet, vol.379, issue.9815, pp.517-518, 2012. ,
DOI : 10.1016/S0140-6736(12)60228-1
Eculizumab in Severe Shiga-Toxin???Associated HUS, New England Journal of Medicine, vol.364, issue.26, pp.2561-2564, 2011. ,
DOI : 10.1056/NEJMc1100859
Complement contributes to the??pathogenesis of Shiga toxin???associated hemolytic uremic syndrome, Kidney International, vol.90, issue.4, pp.726-735, 2016. ,
DOI : 10.1016/j.kint.2016.07.002
Need for Long-term Follow-up in Enterohemorrhagic Escherichia coli???Associated Hemolytic Uremic Syndrome Due to Late-Emerging Sequelae, Clinical Infectious Diseases, vol.54, issue.10, pp.1413-1434, 2012. ,
DOI : 10.1093/cid/cis196
The haemolytic uraemic syndrome--a family study, Q J Med. avr, vol.44, issue.174, pp.161-88, 1975. ,
Hemolytic Uremic Syndrome in Families, New England Journal of Medicine, vol.292, issue.21, pp.1090-1093, 1975. ,
DOI : 10.1056/NEJM197505222922102
Hypocomplementaemia due to a genetic deficiency of beta 1H globulin, Clin Exp Immunol. oct, vol.46, issue.1, pp.110-119, 1981. ,
Genetic studies into inherited and sporadic hemolytic uremic syndrome, Kidney International, vol.53, issue.4, pp.836-880, 1998. ,
DOI : 10.1111/j.1523-1755.1998.00824.x
Complement System Part I ???????? Molecular Mechanisms of Activation and Regulation, Frontiers in Immunology, vol.703, issue.181, p.262, 2015. ,
DOI : 10.1007/978-1-4419-5635-4_10
Atypical haemolytic uraemic syndrome and mutations in complement regulator genes, Springer Seminars in Immunopathology, vol.78, issue.11, pp.359-74, 2005. ,
DOI : 10.4049/jimmunol.169.4.2102
Glomerular Diseases Dependent on Complement Activation, Including Atypical Hemolytic Uremic Syndrome, Membranoproliferative Glomerulonephritis, and C3 Glomerulopathy: Core Curriculum 2015, American Journal of Kidney Diseases, vol.66, issue.2, pp.359-75, 2015. ,
DOI : 10.1053/j.ajkd.2015.03.040
Differential Impact of Complement Mutations on Clinical Characteristics in Atypical Hemolytic Uremic Syndrome, Journal of the American Society of Nephrology, vol.18, issue.8, pp.2392-400, 2007. ,
DOI : 10.1681/ASN.2006080811
URL : https://hal.archives-ouvertes.fr/inserm-00409786
Relative Role of Genetic Complement Abnormalities in Sporadic and Familial aHUS and Their Impact on Clinical Phenotype, Clinical Journal of the American Society of Nephrology, vol.5, issue.10, pp.1844-59, 2010. ,
DOI : 10.2215/CJN.02210310
Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome, Blood, vol.108, issue.4, pp.1267-79, 2006. ,
DOI : 10.1182/blood-2005-10-007252
Pregnancy-Associated Hemolytic Uremic Syndrome Revisited in the Era of Complement Gene Mutations, Journal of the American Society of Nephrology, vol.21, issue.5, pp.859-67, 2010. ,
DOI : 10.1681/ASN.2009070706
URL : https://hal.archives-ouvertes.fr/hal-00512540
Atypical hemolytic uremic syndrome, Orphanet Journal of Rare Diseases, vol.6, issue.1, p.60, 2011. ,
DOI : 10.1007/s00467-010-1608-9
URL : https://hal.archives-ouvertes.fr/hal-01064383
Clinical Features of Anti-Factor H Autoantibody-Associated Hemolytic Uremic Syndrome, Journal of the American Society of Nephrology, vol.21, issue.12, pp.2180-2187, 2010. ,
DOI : 10.1681/ASN.2010030315
Treatment of the hemolytic uremic syndrome with plasma, Clin Nephrol. déc, vol.12, issue.6, pp.279-84, 1979. ,
Terminal Complement Inhibitor Eculizumab in Atypical Hemolytic???Uremic Syndrome, New England Journal of Medicine, vol.368, issue.23, pp.2169-81, 2013. ,
DOI : 10.1056/NEJMoa1208981
Advances in the diagnosis and therapy of paroxysmal nocturnal hemoglobinuria, Blood Reviews, vol.22, issue.2, pp.65-74, 2008. ,
DOI : 10.1016/j.blre.2007.10.002
Atypical haemolytic uraemic syndrome and pregnancy: outcome with ongoing eculizumab, Nephrology Dialysis Transplantation, vol.31, issue.12, pp.2122-2152, 2016. ,
DOI : 10.1093/ndt/gfw314
Post-partum atypical haemolytic-uraemic syndrome treated with eculizumab: terminal complement activity assessment in clinical practice, Clinical Kidney Journal, vol.6, issue.2, pp.243-247, 2013. ,
DOI : 10.1093/ckj/sfs185
Genetic Variants in C5 and Poor Response to Eculizumab, New England Journal of Medicine, vol.370, issue.7, pp.632-641, 2014. ,
DOI : 10.1056/NEJMoa1311084
Gain-of-function mutations in complement factor B are associated with atypical hemolytic uremic syndrome, Proceedings of the National Academy of Sciences, vol.277, issue.2, pp.240-245, 2007. ,
DOI : 10.1074/jbc.M109322200
Eculizumab for Atypical Hemolytic Uremic Syndrome Recurrence in Renal Transplantation, American Journal of Transplantation, vol.1, issue.12, pp.3337-54, 2012. ,
DOI : 10.1111/j.1365-2141.2009.07916.x
Haemolytic uraemic syndrome, The Lancet, vol.390, issue.10095, 2017. ,
DOI : 10.1016/S0140-6736(17)30062-4
Syndromes of thrombotic microangiopathy associated with pregnancy, Hematology, vol.2015, issue.1, pp.644-652, 2015. ,
DOI : 10.1182/asheducation-2015.1.644
Renal Cortical Necrosis in Postpartum Hemorrhage: A Case Series, American Journal of Kidney Diseases, vol.68, issue.1, pp.50-57, 2016. ,
DOI : 10.1053/j.ajkd.2015.11.022
Drug-induced thrombotic microangiopathy: a systematic review of published reports, Blood, vol.125, issue.4, pp.616-624, 2015. ,
DOI : 10.1182/blood-2014-11-611335
Association Between Thrombotic Microangiopathy and Reduced ADAMTS13 Activity in Malignant Hypertension, Hypertension, vol.51, issue.4, pp.862-868, 1979. ,
DOI : 10.1161/HYPERTENSIONAHA.107.103127
Patients with hypertension-associated thrombotic??microangiopathy may present with??complement abnormalities, Kidney International, vol.91, issue.6, 2017. ,
DOI : 10.1016/j.kint.2016.12.009
Scleroderma Renal Crisis: A Rare but Severe Complication of Systemic Sclerosis, Clinical Reviews in Allergy & Immunology, vol.66, issue.7, pp.84-91, 2011. ,
DOI : 10.1136/ard.2006.066068
Microangiopathic Hemolytic Anemia and Thrombocytopenia in Patients With Cancer, Journal of Oncology Practice, vol.12, issue.6, pp.523-553, 2016. ,
DOI : 10.1200/JOP.2016.012096
Malignancy and thrombotic microangiopathy or atypical haemolytic and uraemic syndrome?, British Journal of Haematology, vol.8, issue.5, pp.802-807, 2014. ,
DOI : 10.1038/nrneph.2012.214
Diagnostic criteria for hematopoietic stem cell transplant-associated microangiopathy: results of a consensus process by an International Working Group, Haematologica, vol.92, issue.1, pp.95-100, 2007. ,
DOI : 10.3324/haematol.10699
Adult-Onset Eculizumab-Resistant Hemolytic Uremic Syndrome Associated With Cobalamin C Deficiency, American Journal of Kidney Diseases, vol.63, issue.1, pp.119-142, 2014. ,
DOI : 10.1053/j.ajkd.2013.08.031
URL : https://hal.archives-ouvertes.fr/hal-01011789
Pancreatitis preceding acute episodes of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: report of five patients with a systematic review of published reports, Haematologica, vol.92, issue.7, pp.936-979, 2007. ,
DOI : 10.3324/haematol.10963
Severe vitamin B12 deficiency mimicking thrombotic thrombocytopenic purpura, Blood, vol.124, issue.11, p.1844, 2014. ,
DOI : 10.1182/blood-2014-03-562488
Hemolysis, elevated liver enzymes and low platelets during pregnancy due to Vitamin B12 and folate deficiencies, European Journal of Obstetrics & Gynecology and Reproductive Biology, vol.131, issue.2, pp.241-243, 2007. ,
DOI : 10.1016/j.ejogrb.2006.04.026
The thrombotic thrombocytopenic purpura and hemolytic uremic syndromes: evaluation, management, and long-term outcomes experience of the Oklahoma TTP-HUS Registry, Kidney Int Suppl. févr, issue.112, pp.52-54, 1989. ,
Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura, Blood, vol.112, issue.1, pp.11-19, 2008. ,
DOI : 10.1182/blood-2008-02-078170
Severe ADAMTS13 Deficiency in Adult Idiopathic Thrombotic Microangiopathies Defines a Subset of Patients Characterized by Various Autoimmune Manifestations, Lower Platelet Count, and Mild Renal Involvement, Medicine, vol.83, issue.4, pp.233-277, 2004. ,
DOI : 10.1097/01.md.0000133622.03370.07
Pathogenic Variants in Complement Genes and Risk of Atypical Hemolytic Uremic Syndrome Relapse after Eculizumab Discontinuation, Clinical Journal of the American Society of Nephrology, vol.12, issue.1, pp.50-59, 2017. ,
DOI : 10.2215/CJN.06440616
Successful treatment with rituximab for acute refractory thrombotic thrombocytopenic purpura related to acquired ADAMTS13 deficiency: a pediatric report and literature review. Pediatr Crit Care Med J Soc Crit Care Med World Fed Pediatr Intensive Crit Care Soc, mars, vol.12, issue.2, pp.90-93, 2011. ,
Clinical features of severe acquired ADAMTS13 deficiency in thrombotic thrombocytopenic purpura: the Korean TTP registry experience, International Journal of Hematology, vol.93, issue.Suppl 2, pp.163-172, 2011. ,
DOI : 10.3324/haematol.11739
Registry of 919 Patients with Thrombotic Microangiopathies across Japan: Database of Nara Medical University during 1998-2008, Internal Medicine, vol.49, issue.1, pp.7-15, 2010. ,
DOI : 10.2169/internalmedicine.49.2706
Genetics and Outcome of Atypical Hemolytic Uremic Syndrome: A Nationwide French Series Comparing Children and Adults, Clinical Journal of the American Society of Nephrology, vol.8, issue.4, pp.554-62, 2013. ,
DOI : 10.2215/CJN.04760512
Nous avons inclus rétrospectivement tous les patients âgés de plus de 15 ans et 3 ,
Parmi les 94 patients inclus, 42,5% avaient un PTT acquis et 18, p.1 ,
5%) et à plus d'atteinte neurologique (31%) Parmi eux, 50% ont reçu un traitement immunosuppresseur en première ligne et 32,5% en traitement d'entretien, associé à seulement 9,4% de rechute clinique mais un taux de mortalité élevé de 20%. Le CM-HUS était significativement associé à plus d'atteinte rénale sévère (70.6%) Parmi eux, 76,5% ont reçu de l'Eculizumab, associé à un taux de rechute clinique de 30% mais seulement 7,1% d'insuffisance rénale chronique stade V à la fin du suivi ,