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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

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
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
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

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
Michael M, Bagga A, Sartain SE, Smith RJH
Lancet 2022 Nov 12;400(10364):1722-1740. Epub 2022 Oct 19 doi: 10.1016/S0140-6736(22)01202-8. PMID: 36272423
Sheerin NS, Glover E
F1000Res 2019;8 Epub 2019 Sep 25 doi: 10.12688/f1000research.19957.1. PMID: 31598213Free PMC Article
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
Brocklebank V, Wood KM, Kavanagh D
Clin J Am Soc Nephrol 2018 Feb 7;13(2):300-317. Epub 2017 Oct 17 doi: 10.2215/CJN.00620117. PMID: 29042465Free PMC Article

Diagnosis

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
Michael M, Bagga A, Sartain SE, Smith RJH
Lancet 2022 Nov 12;400(10364):1722-1740. Epub 2022 Oct 19 doi: 10.1016/S0140-6736(22)01202-8. PMID: 36272423
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
Palma LMP, Vaisbich-Guimarães MH, Sridharan M, Tran CL, Sethi S
Pediatr Nephrol 2022 Sep;37(9):1967-1980. Epub 2022 Jan 18 doi: 10.1007/s00467-021-05370-8. PMID: 35041041Free PMC Article
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

Therapy

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
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
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
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
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

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
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
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
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
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

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
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
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
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
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

Chen C, Li Y, Shi P, Qian S
Blood Cancer J 2024 Nov 7;14(1):196. doi: 10.1038/s41408-024-01182-9. PMID: 39511171Free PMC Article
Arhip L, Brox-Torrecilla N, Romero I, Motilla M, Serrano-Moreno C, Miguélez M, Cuerda C
Orphanet J Rare Dis 2024 Jan 20;19(1):20. doi: 10.1186/s13023-024-03021-3. PMID: 38245797Free PMC Article
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
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
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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