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Thrombotic microangiopathy

MedGen UID:
403479
•Concept ID:
C2717961
•
Disease or Syndrome
Synonyms: Microangiopathies, Thrombotic; Microangiopathy, Thrombotic; Thrombotic Microangiopathies; Thrombotic Microangiopathy
SNOMED CT: Thrombotic microangiopathy (126729006)
 
Monarch Initiative: MONDO:0019737
Orphanet: ORPHA93573

Definition

A microvascular coagulopathy that may result from systemic vascular endothelial injury triggering the development of a procoagulant state, activation of the complement cascade, and microthrombi formation. Signs may include hemolytic anemia, thrombocytopenia, hypertension and renal dysfunction. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVThrombotic microangiopathy
Follow this link to review classifications for Thrombotic microangiopathy in Orphanet.

Professional guidelines

PubMed

Clinical Evaluation and Management of Thrombotic Microangiopathy.
Leisring J, Brodsky SV, Parikh SV
Arthritis Rheumatol 2024 Feb;76(2):153-165. Epub 2023 Nov 30 doi: 10.1002/art.42681. PMID: 37610060
Diagnosis and treatment of thrombotic microangiopathy.
Thompson GL, Kavanagh D
Int J Lab Hematol 2022 Sep;44 Suppl 1(Suppl 1):101-113. doi: 10.1111/ijlh.13954. PMID: 36074708Free PMC Article
Atypical Hemolytic-Uremic Syndrome: An Update on Pathophysiology, Diagnosis, and Treatment.
Raina R, Krishnappa V, Blaha T, Kann T, Hein W, Burke L, Bagga A
Ther Apher Dial 2019 Feb;23(1):4-21. Epub 2018 Oct 29 doi: 10.1111/1744-9987.12763. PMID: 30294946

Recent clinical studies

Etiology

Treatment outcome and efficacy of therapeutic plasma exchange for transplant-associated thrombotic microangiopathy in a large real-world cohort study.
Yang LP, Zhao P, Wu YJ, Fu HX, He Y, Mo XD, Lv M, Wang FR, Yan CH, Chen YH, Chang YJ, Xu LP, Liu KY, Huang XJ, Zhang XH
Bone Marrow Transplant 2022 Apr;57(4):554-561. Epub 2022 Jan 25 doi: 10.1038/s41409-022-01581-1. PMID: 35079139

Therapy

Harmonizing Definitions for Diagnostic Criteria and Prognostic Assessment of Transplantation-Associated Thrombotic Microangiopathy: A Report on Behalf of the European Society for Blood and Marrow Transplantation, American Society for Transplantation and Cellular Therapy, Asia-Pacific Blood and Marrow Transplantation Group, and Center for International Blood and Marrow Transplant Research.
Schoettler ML, Carreras E, Cho B, Dandoy CE, Ho VT, Jodele S, Moissev I, Sanchez-Ortega I, Srivastava A, Atsuta Y, Carpenter P, Koreth J, Kroger N, Ljungman P, Page K, Popat U, Shaw BE, Sureda A, Soiffer R, Vasu S
Transplant Cell Ther 2023 Mar;29(3):151-163. Epub 2022 Nov 25 doi: 10.1016/j.jtct.2022.11.015. PMID: 36442770Free PMC Article
Transplant-associated thrombotic microangiopathy: theoretical considerations and a practical approach to an unrefined diagnosis.
Young JA, Pallas CR, Knovich MA
Bone Marrow Transplant 2021 Aug;56(8):1805-1817. Epub 2021 Apr 19 doi: 10.1038/s41409-021-01283-0. PMID: 33875812Free PMC Article
Therapeutic Inhibition of VEGF Signaling and Associated Nephrotoxicities.
Estrada CC, Maldonado A, Mallipattu SK
J Am Soc Nephrol 2019 Feb;30(2):187-200. Epub 2019 Jan 14 doi: 10.1681/ASN.2018080853. PMID: 30642877Free PMC Article
A new paradigm: Diagnosis and management of HSCT-associated thrombotic microangiopathy as multi-system endothelial injury.
Jodele S, Laskin BL, Dandoy CE, Myers KC, El-Bietar J, Davies SM, Goebel J, Dixon BP
Blood Rev 2015 May;29(3):191-204. Epub 2014 Nov 28 doi: 10.1016/j.blre.2014.11.001. PMID: 25483393Free PMC Article
Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome.
Legendre CM, Licht C, Muus P, Greenbaum LA, Babu S, Bedrosian C, Bingham C, Cohen DJ, Delmas Y, Douglas K, Eitner F, Feldkamp T, Fouque D, Furman RR, Gaber O, Herthelius M, Hourmant M, Karpman D, Lebranchu Y, Mariat C, Menne J, Moulin B, Nürnberger J, Ogawa M, Remuzzi G, Richard T, Sberro-Soussan R, Severino B, Sheerin NS, Trivelli A, Zimmerhackl LB, Goodship T, Loirat C
N Engl J Med 2013 Jun 6;368(23):2169-81. doi: 10.1056/NEJMoa1208981. PMID: 23738544

Prognosis

Hemolytic-Uremic Syndrome in Children.
Boyer O, Niaudet P
Pediatr Clin North Am 2022 Dec;69(6):1181-1197. Epub 2022 Oct 29 doi: 10.1016/j.pcl.2022.07.006. PMID: 36880929
An update on pathogenesis and diagnosis of thrombotic thrombocytopenic purpura.
Joly BS, Coppo P, Veyradier A
Expert Rev Hematol 2019 Jun;12(6):383-395. Epub 2019 May 20 doi: 10.1080/17474086.2019.1611423. PMID: 31107120
Etiology and Outcomes of Thrombotic Microangiopathies.
Bayer G, von Tokarski F, Thoreau B, Bauvois A, Barbet C, Cloarec S, Mérieau E, Lachot S, Garot D, Bernard L, Gyan E, Perrotin F, Pouplard C, Maillot F, Gatault P, Sautenet B, Rusch E, Buchler M, Vigneau C, Fakhouri F, Halimi JM
Clin J Am Soc Nephrol 2019 Apr 5;14(4):557-566. Epub 2019 Mar 12 doi: 10.2215/CJN.11470918. PMID: 30862697Free PMC Article
Thrombotic microangiopathies of pregnancy: Differential diagnosis.
Gupta M, Feinberg BB, Burwick RM
Pregnancy Hypertens 2018 Apr;12:29-34. Epub 2018 Feb 16 doi: 10.1016/j.preghy.2018.02.007. PMID: 29674195
A new paradigm: Diagnosis and management of HSCT-associated thrombotic microangiopathy as multi-system endothelial injury.
Jodele S, Laskin BL, Dandoy CE, Myers KC, El-Bietar J, Davies SM, Goebel J, Dixon BP
Blood Rev 2015 May;29(3):191-204. Epub 2014 Nov 28 doi: 10.1016/j.blre.2014.11.001. PMID: 25483393Free PMC Article

Clinical prediction guides

Renal Thrombotic Microangiopathy: A Review.
Genest DS, Patriquin CJ, Licht C, John R, Reich HN
Am J Kidney Dis 2023 May;81(5):591-605. Epub 2022 Dec 10 doi: 10.1053/j.ajkd.2022.10.014. PMID: 36509342
Thrombotic microangiopathies of pregnancy: Differential diagnosis.
Gupta M, Feinberg BB, Burwick RM
Pregnancy Hypertens 2018 Apr;12:29-34. Epub 2018 Feb 16 doi: 10.1016/j.preghy.2018.02.007. PMID: 29674195
An international consensus approach to the management of atypical hemolytic uremic syndrome in children.
Loirat C, Fakhouri F, Ariceta G, Besbas N, Bitzan M, Bjerre A, Coppo R, Emma F, Johnson S, Karpman D, Landau D, Langman CB, Lapeyraque AL, Licht C, Nester C, Pecoraro C, Riedl M, van de Kar NC, Van de Walle J, Vivarelli M, Frémeaux-Bacchi V; HUS International
Pediatr Nephrol 2016 Jan;31(1):15-39. Epub 2015 Apr 11 doi: 10.1007/s00467-015-3076-8. PMID: 25859752
Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome.
Legendre CM, Licht C, Muus P, Greenbaum LA, Babu S, Bedrosian C, Bingham C, Cohen DJ, Delmas Y, Douglas K, Eitner F, Feldkamp T, Fouque D, Furman RR, Gaber O, Herthelius M, Hourmant M, Karpman D, Lebranchu Y, Mariat C, Menne J, Moulin B, Nürnberger J, Ogawa M, Remuzzi G, Richard T, Sberro-Soussan R, Severino B, Sheerin NS, Trivelli A, Zimmerhackl LB, Goodship T, Loirat C
N Engl J Med 2013 Jun 6;368(23):2169-81. doi: 10.1056/NEJMoa1208981. PMID: 23738544
Antiphospholipid syndrome.
Ruiz-Irastorza G, Crowther M, Branch W, Khamashta MA
Lancet 2010 Oct 30;376(9751):1498-509. Epub 2010 Sep 6 doi: 10.1016/S0140-6736(10)60709-X. PMID: 20822807

Recent systematic reviews

Risk factors for transplant-associated thrombotic microangiopathy (TA-TMA): a systematic review and meta-analysis.
Guo M, Qi J, Hou Q, Li X, Han Y
Expert Rev Hematol 2023 Mar;16(3):191-203. Epub 2023 Jan 2 doi: 10.1080/17474086.2023.2162501. PMID: 36588482
A systematic review of diagnostic, prognostic, and risk blood and urine biomarkers of transplant-associated thrombotic microangiopathy.
Schoettler ML, Bhatt H, Vasu S
Front Immunol 2022;13:1064203. Epub 2023 Feb 2 doi: 10.3389/fimmu.2022.1064203. PMID: 36818475Free PMC Article
rAAV immunogenicity, toxicity, and durability in 255 clinical trials: A meta-analysis.
Shen W, Liu S, Ou L
Front Immunol 2022;13:1001263. Epub 2022 Oct 27 doi: 10.3389/fimmu.2022.1001263. PMID: 36389770Free PMC Article
Cobalamin deficiency presenting with thrombotic microangiopathy (TMA) features: A systematic review.
Tran PN, Tran MH
Transfus Apher Sci 2018 Feb;57(1):102-106. Epub 2018 Jan 11 doi: 10.1016/j.transci.2018.01.003. PMID: 29454538
Drug-induced thrombotic microangiopathy: a systematic review of published reports.
Al-Nouri ZL, Reese JA, Terrell DR, Vesely SK, George JN
Blood 2015 Jan 22;125(4):616-8. Epub 2014 Nov 20 doi: 10.1182/blood-2014-11-611335. PMID: 25414441Free PMC Article

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