Syndromes of thrombotic microangiopathy.

This review article covers the diverse pathophysiological pathways that can lead to microangiopathic hemolytic anemia and a procoagulant state with or without damage to the kidneys and other organs.

[1]  T. Foroud,et al.  Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura , 2001, Nature.

[2]  S. Vesely,et al.  Evidence for a role of anti-ADAMTS13 autoantibodies despite normal ADAMTS13 activity in recurrent thrombotic thrombocytopenic purpura , 2012, Haematologica.

[3]  É. Azoulay,et al.  Unexpected frequency of Upshaw-Schulman syndrome in pregnancy-onset thrombotic thrombocytopenic purpura. , 2012, Blood.

[4]  E. Markell,et al.  Blastocystis hominis: prevalence in asymptomatic versus symptomatic hosts. , 1993, The Journal of infectious diseases.

[5]  D. Mahoney,et al.  Successful Treatment of Congenital TTP With a Novel Approach Using Plasma-derived Factor VIII , 2013, Journal of pediatric hematology/oncology.

[6]  P. Stricklett,et al.  Molecular basis for high renal cell sensitivity to the cytotoxic effects of shigatoxin-1: upregulation of globotriaosylceramide expression. , 2002, Journal of the American Society of Nephrology : JASN.

[7]  F. Clayton,et al.  Shigatoxin-1 binding and receptor expression in human kidneys do not change with age , 2003, Pediatric Nephrology.

[8]  R. Gibbs,et al.  Residual plasmatic activity of ADAMTS13 is correlated with phenotype severity in congenital thrombotic thrombocytopenic purpura. , 2012, Blood.

[9]  S. Vesely,et al.  Sporadic bloody diarrhoea‐associated thrombotic thrombocytopenic purpura‐haemolytic uraemic syndrome: an adult and paediatric comparison , 2008, British journal of haematology.

[10]  D. Lau,et al.  Thrombotic Thrombocytopenic Purpura Associated with Quetiapine , 2005, The Annals of pharmacotherapy.

[11]  J. Stockman Thrombomodulin Mutations in Atypical Hemolytic–Uremic Syndrome , 2011 .

[12]  A. Losito,et al.  Combined Pulmonary Hypertension and Renal Thrombotic Microangiopathy in Cobalamin C Deficiency , 2013, Pediatrics.

[13]  Richard J. H. Smith,et al.  Comprehensive genetic analysis of complement and coagulation genes in atypical hemolytic uremic syndrome. , 2014, Journal of the American Society of Nephrology : JASN.

[14]  P. Birembaut,et al.  Sirolimus‐Induced Thrombotic Microangiopathy is Associated with Decreased Expression of Vascular Endothelial Growth Factor in Kidneys , 2005, American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons.

[15]  R. Thompson,et al.  Hypocomplementaemia due to a genetic deficiency of beta 1H globulin. , 1981, Clinical and experimental immunology.

[16]  J. George,et al.  How I treat patients with thrombotic thrombocytopenic purpura: 2010. , 2010, Blood.

[17]  R. Aster,et al.  Quinine‐induced immune thrombocytopenia with hemolytic uremic syndrome: Clinical and serological findings in nine patients and review of literature , 1994, American journal of hematology.

[18]  W. D. de Vos,et al.  The microbial eukaryote Blastocystis is a prevalent and diverse member of the healthy human gut microbiota. , 2014, FEMS microbiology ecology.

[19]  G. Remuzzi,et al.  Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. , 2013, The New England journal of medicine.

[20]  H. Pennington,et al.  Escherichia coli O157 , 2010, The Lancet.

[21]  S. Vesely,et al.  Quinine-Associated Thrombotic Thrombocytopenic PurpuraHemolytic Uremic Syndrome: Frequency, Clinical Features, and Long-Term Outcomes , 2001, Annals of Internal Medicine.

[22]  Deborah A. Adams,et al.  Summary of Notifiable Infectious Diseases and Conditions - United States, 2013. , 2015, MMWR. Morbidity and mortality weekly report.

[23]  D. Deykin,et al.  Unusually large plasma factor VIII:von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura. , 1982, The New England journal of medicine.

[24]  S. Quaggin DGKE and atypical HUS , 2013, Nature Genetics.

[25]  W. Herrmann,et al.  Homocysteine, Cystathionine, Methylmalonic Acid and B-Vitamins in Patients with Renal Disease , 2001, Clinical chemistry and laboratory medicine.

[26]  M. Rahaman,et al.  Shiga bacillus dysentery associated with marked leukocytosis and erythrocyte fragmentation. , 1975, The Johns Hopkins medical journal.

[27]  D. Motto,et al.  Shiga toxin B subunits induce VWF secretion by human endothelial cells and thrombotic microangiopathy in ADAMTS13-deficient mice. , 2010, Blood.

[28]  G. Hutchins,et al.  Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are distinct pathologic entities. A review of 56 autopsy cases. , 2003, Archives of pathology & laboratory medicine.

[29]  A. Chauhan,et al.  ADAMTS13 reduces vascular inflammation and the development of early atherosclerosis in mice. , 2012, Blood.

[30]  J. Racoosin,et al.  Thrombotic Thrombocytopenic Purpura (TTP)–Like Illness Associated with Intravenous Opana ER Abuse — Tennessee, 2012 , 2013, MMWR. Morbidity and mortality weekly report.

[31]  D. Maki Don't eat the spinach--controlling foodborne infectious disease. , 2006, The New England journal of medicine.

[32]  B. Lämmle,et al.  Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. , 1996, Blood.

[33]  A. Salama,et al.  Quinine-induced immune thrombocytopenic purpura followed by hemolytic uremic syndrome. , 1999, American journal of kidney diseases : the official journal of the National Kidney Foundation.

[34]  James G. Scott,et al.  Cognitive deficits after recovery from thrombotic thrombocytopenic purpura , 2009, Transfusion.

[35]  J. Wells,et al.  A multistate outbreak of Escherichia coli O157:H7-associated bloody diarrhea and hemolytic uremic syndrome from hamburgers. The Washington experience. , 1994, JAMA.

[36]  M. Petric,et al.  SPORADIC CASES OF HAEMOLYTIC-URAEMIC SYNDROME ASSOCIATED WITH FAECAL CYTOTOXIN AND CYTOTOXIN-PRODUCING ESCHERICHIA COLI IN STOOLS , 1983, The Lancet.

[37]  J. George,et al.  Thrombotic thrombocytopenic purpura. Occurrence two years apart during late pregnancy in two sisters. , 1976, JAMA.

[38]  D. Nochy,et al.  VEGF inhibition and renal thrombotic microangiopathy. , 2008, The New England journal of medicine.

[39]  D. Valle,et al.  Cobalamin C defect associated with hemolytic-uremic syndrome. , 1992, The Journal of pediatrics.

[40]  M. Ciol,et al.  Prothrombotic coagulation abnormalities preceding the hemolytic-uremic syndrome. , 2002, The New England journal of medicine.

[41]  S. Vesely,et al.  Children and adults with thrombotic thrombocytopenic purpura associated with severe, acquired Adamts13 deficiency: Comparison of incidence, demographic and clinical features , 2013, Pediatric blood & cancer.

[42]  R. Chesney,et al.  Hemolytic uremic syndrome in families. , 1975, The New England journal of medicine.

[43]  S. Testa,et al.  Neonatal atypical hemolytic uremic syndrome due to methylmalonic aciduria and homocystinuria , 2012, Pediatric Nephrology.

[44]  H. Tsai Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. , 1996, Blood.

[45]  J. Ultmann,et al.  THROMBOTIC THROMBOCYTOPENIC PURPURA: REPORT OF 16 CASES AND REVIEW OF THE LITERATURE , 1966 .

[46]  B. Senior,et al.  Syndrome of hemolysis, thrombopenia and nephropathy in infancy. , 1962, Pediatrics.

[47]  I. Edwards,et al.  Adverse drug reactions: definitions, diagnosis, and management , 2000, The Lancet.

[48]  Rahaman Mm,et al.  Shiga bacillus dysentery associated with marked leukocytosis and erythrocyte fragmentation. , 1975 .

[49]  A. Morley,et al.  The haemolytic uraemic syndrome--a family study. , 1975, The Quarterly journal of medicine.

[50]  J. Kelton,et al.  Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. , 1991, The New England journal of medicine.

[51]  G. Davı̀,et al.  Homocysteine, Coagulation, Platelet Function, and Thrombosis , 2000, Seminars in thrombosis and hemostasis.

[52]  J. Stockman Treatment of severe neurological deficits with IgG depletion through immunoadsorption in patients with Escherichia coli O104:H4-associated haemolytic uraemic syndrome: a prospective trial , 2013 .

[53]  B. Lämmle,et al.  Recovery and Half-Life of von Willebrand Factor-Cleaving Protease after Plasma Therapy in Patients with Thrombotic Thrombocytopenic Purpura , 1999, Thrombosis and Haemostasis.

[54]  J. George How I treat patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. , 2000, Blood.

[55]  G. Remuzzi,et al.  Hemolytic Uremic Syndrome/Thrombotic Thrombocytopenic Purpura , 2010 .

[56]  T. Yutsudo,et al.  Site of action of a Vero toxin (VT2) from Escherichia coli O157:H7 and of Shiga toxin on eukaryotic ribosomes. RNA N-glycosidase activity of the toxins. , 1988, European journal of biochemistry.

[57]  J. Goodship,et al.  Genetic studies into inherited and sporadic hemolytic uremic syndrome. , 1998, Kidney international.

[58]  R. Aster,et al.  Patients with quinine-induced immune thrombocytopenia have both "drug-dependent" and "drug-specific" antibodies. , 2006, Blood.

[59]  H. Karch,et al.  Need for long-term follow-up in enterohemorrhagic Escherichia coli-associated hemolytic uremic syndrome due to late-emerging sequelae. , 2012, Clinical infectious diseases : an official publication of the Infectious Diseases Society of America.

[60]  E. Moschcowitz Hyaline thrombosis of the terminal arterioles and capillaries : a hitherto undescribed disease , 1924 .

[61]  J. Doyon,et al.  A congenital anomaly of vitamin B12 metabolism: a study of three cases. , 1992, Human pathology.

[62]  S. Vesely,et al.  Survival and relapse in patients with thrombotic thrombocytopenic purpura. , 2010, Blood.

[63]  J. Inazawa,et al.  Genetic variants in C5 and poor response to eculizumab. , 2014, The New England journal of medicine.

[64]  S. Vesely,et al.  Multiple major morbidities and increased mortality during long-term follow-up after recovery from thrombotic thrombocytopenic purpura. , 2013, Blood.

[65]  Hocken Ag,et al.  Acute intravascular haemolysis due to quinine. , 1980 .

[66]  P. Tarr,et al.  Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome , 2005, The Lancet.

[67]  Matthias Greiner,et al.  German outbreak of Escherichia coli O104:H4 associated with sprouts. , 2011, The New England journal of medicine.

[68]  P. Zipfel,et al.  Factor H dysfunction in patients with atypical hemolytic uremic syndrome contributes to complement deposition on platelets and their activation. , 2008, Blood.

[69]  J. George,et al.  Ten patient stories illustrating the extraordinarily diverse clinical features of patients with thrombotic thrombocytopenic purpura and severe ADAMTS13 deficiency , 2012, Journal of clinical apheresis.

[70]  T. Miyata,et al.  Natural history of Upshaw–Schulman syndrome based on ADAMTS13 gene analysis in Japan , 2011, Journal of thrombosis and haemostasis : JTH.

[71]  Harry B. McGee,et al.  Hemorrhagic colitis associated with a rare Escherichia coli serotype , 1983 .

[72]  C. Lingwood,et al.  Escherichia coli verotoxin binding to human paediatric glomerular mesangial cells , 1995, Pediatric Nephrology.

[73]  A. Garg,et al.  Long-term renal prognosis of diarrhea-associated hemolytic uremic syndrome: a systematic review, meta-analysis, and meta-regression. , 2003, JAMA.

[74]  John Hwa,et al.  Recessive mutations in DGKE cause atypical hemolytic-uremic syndrome , 2013, Nature Genetics.

[75]  S. Bigler,et al.  Hemolytic uremic syndrome associated with group A beta-hemolytic streptococcus , 2003, Pediatric Nephrology.

[76]  A. Chauhan,et al.  Shigatoxin triggers thrombotic thrombocytopenic purpura in genetically susceptible ADAMTS13-deficient mice. , 2005, The Journal of clinical investigation.

[77]  Lawrence Copelovitch,et al.  Streptococcus pneumoniae–Associated Hemolytic Uremic Syndrome: Classification and the Emergence of Serotype 19A , 2010, Pediatrics.

[78]  L. Doucet,et al.  Adult-onset eculizumab-resistant hemolytic uremic syndrome associated with cobalamin C deficiency. , 2014, American journal of kidney diseases : the official journal of the National Kidney Foundation.

[79]  S. Katz,et al.  Questionable clinical significance of Blastocystis hominis infection. , 1989, The American journal of gastroenterology.

[80]  G. Remuzzi,et al.  Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype. , 2010, Clinical journal of the American Society of Nephrology : CJASN.

[81]  M. Saif,et al.  Thrombotic microangiopathy associated with gemcitabine: rare but real , 2009, Expert opinion on drug safety.

[82]  P. Sax,et al.  Association Between Efavirenz as Initial Therapy for HIV-1 Infection and Increased Risk for Suicidal Ideation or Attempted or Completed Suicide , 2014, Annals of Internal Medicine.

[83]  P. Merkel,et al.  2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. , 2013, Arthritis and rheumatism.

[84]  A. Bakkaloğlu,et al.  DGKE variants cause a glomerular microangiopathy that mimics membranoproliferative GN. , 2013, Journal of the American Society of Nephrology : JASN.

[85]  A. Steck,et al.  [Hemolytic-uremic syndrome: bilateral necrosis of the renal cortex in acute acquired hemolytic anemia]. , 1955, Schweizerische medizinische Wochenschrift.

[86]  C. Greenberg,et al.  Hemolytic uremic syndrome (HUS) secondary to cobalamin C (cblC) disorder , 2007, Pediatric Nephrology.

[87]  J. Moake Thrombotic microangiopathies. , 2002, The New England journal of medicine.

[88]  M. Baumgartner,et al.  Clinical presentation and outcome in a series of 88 patients with the cblC defect , 2014, Journal of Inherited Metabolic Disease.

[89]  栄一 末松 血管炎の新分類2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides , 2013 .