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Acquired thrombotic thrombocytopenic purpura

MedGen UID:
391723
Concept ID:
C2584778
Disease or Syndrome
Synonym: Idiopathic thrombotic thrombocytopenic purpura
SNOMED CT: Acquired thrombotic thrombocytopenic purpura (439007008); Acquired thrombotic thrombocytopenic purpura (TTP) (439007008)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
Concept ID:
C0600599
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
 
Monarch Initiative: MONDO:0019740
Orphanet: ORPHA93585

Definition

A rare, non-hereditary thrombotic thrombocytopenic purpura (TTP), characterized by profound peripheral thrombocytopenia, microangiopathic hemolytic anemia (MAHA) and single or multiple organ failure of variable severity. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAcquired thrombotic thrombocytopenic purpura
Follow this link to review classifications for Acquired thrombotic thrombocytopenic purpura in Orphanet.

Professional guidelines

PubMed

Zini G, De Cristofaro R
Turk J Haematol 2019 Nov 18;36(4):222-229. Epub 2019 Jul 24 doi: 10.4274/tjh.galenos.2019.2019.0165. PMID: 31337190Free PMC Article
Elverdi T, Eskazan AE
Drug Des Devel Ther 2019;13:1251-1258. Epub 2019 Apr 17 doi: 10.2147/DDDT.S134470. PMID: 31118566Free PMC Article
Cataland SR, Wu HM
Blood Rev 2014 Mar;28(2):67-74. Epub 2014 Jan 31 doi: 10.1016/j.blre.2014.01.003. PMID: 24534133

Recent clinical studies

Etiology

Elverdi T, Eskazan AE
Drug Des Devel Ther 2019;13:1251-1258. Epub 2019 Apr 17 doi: 10.2147/DDDT.S134470. PMID: 31118566Free PMC Article
Scully M, Cataland SR, Peyvandi F, Coppo P, Knöbl P, Kremer Hovinga JA, Metjian A, de la Rubia J, Pavenski K, Callewaert F, Biswas D, De Winter H, Zeldin RK; HERCULES Investigators
N Engl J Med 2019 Jan 24;380(4):335-346. Epub 2019 Jan 9 doi: 10.1056/NEJMoa1806311. PMID: 30625070
Peyvandi F, Scully M, Kremer Hovinga JA, Cataland S, Knöbl P, Wu H, Artoni A, Westwood JP, Mansouri Taleghani M, Jilma B, Callewaert F, Ulrichts H, Duby C, Tersago D; TITAN Investigators
N Engl J Med 2016 Feb 11;374(6):511-22. doi: 10.1056/NEJMoa1505533. PMID: 26863353
Vesely SK
J Thromb Haemost 2015 Jun;13 Suppl 1:S216-22. doi: 10.1111/jth.12953. PMID: 26149027
Knöbl P
Semin Thromb Hemost 2014 Jun;40(4):493-502. Epub 2014 May 6 doi: 10.1055/s-0034-1376883. PMID: 24802084

Diagnosis

Tanhehco YC, Arepally G, Metjian A
Curr Opin Hematol 2017 Nov;24(6):521-528. doi: 10.1097/MOH.0000000000000375. PMID: 28759473
Cataland SR, Wu HM
J Thromb Haemost 2015 Jun;13 Suppl 1:S223-9. doi: 10.1111/jth.12934. PMID: 26149028
Vesely SK
J Thromb Haemost 2015 Jun;13 Suppl 1:S216-22. doi: 10.1111/jth.12953. PMID: 26149027
Knöbl P
Semin Thromb Hemost 2014 Jun;40(4):493-502. Epub 2014 May 6 doi: 10.1055/s-0034-1376883. PMID: 24802084
Cataland SR, Wu HM
Blood Rev 2014 Mar;28(2):67-74. Epub 2014 Jan 31 doi: 10.1016/j.blre.2014.01.003. PMID: 24534133

Therapy

Scully M, Cataland SR, Peyvandi F, Coppo P, Knöbl P, Kremer Hovinga JA, Metjian A, de la Rubia J, Pavenski K, Callewaert F, Biswas D, De Winter H, Zeldin RK; HERCULES Investigators
N Engl J Med 2019 Jan 24;380(4):335-346. Epub 2019 Jan 9 doi: 10.1056/NEJMoa1806311. PMID: 30625070
Tanhehco YC, Arepally G, Metjian A
Curr Opin Hematol 2017 Nov;24(6):521-528. doi: 10.1097/MOH.0000000000000375. PMID: 28759473
Peyvandi F, Scully M, Kremer Hovinga JA, Cataland S, Knöbl P, Wu H, Artoni A, Westwood JP, Mansouri Taleghani M, Jilma B, Callewaert F, Ulrichts H, Duby C, Tersago D; TITAN Investigators
N Engl J Med 2016 Feb 11;374(6):511-22. doi: 10.1056/NEJMoa1505533. PMID: 26863353
Cataland SR, Wu HM
J Thromb Haemost 2015 Jun;13 Suppl 1:S223-9. doi: 10.1111/jth.12934. PMID: 26149028
Knöbl P
Semin Thromb Hemost 2014 Jun;40(4):493-502. Epub 2014 May 6 doi: 10.1055/s-0034-1376883. PMID: 24802084

Prognosis

Del Río-Garma J, Bobillo S, de la Rubia J, Pascual C, García-Candel F, García-Gala JM, Gonzalez R, Abril L, Vidan J, Gomez MJ, Peña F, Arbona C, Martín-Sanchez J, Moreno G, Romón I, Viejo A, Oliva A, Linares M, Salinas R, Pérez S, Garcia-Erce JA, Pereira A; “Registro Español de la Púrpura Trombocitopénica Trombótica (REPTT)”, and the “Grupo Español de Aféresis (GEA)”
Ann Hematol 2022 Jan;101(1):59-67. Epub 2021 Oct 12 doi: 10.1007/s00277-021-04685-8. PMID: 34642787
Sun RX, Xu J, Zhu HD, Yu XZ, Yang J
Ther Apher Dial 2021 Feb;25(1):118-123. Epub 2020 May 4 doi: 10.1111/1744-9987.13502. PMID: 32306556
Völker LA, Brinkkoetter PT, Knöbl PN, Krstic M, Kaufeld J, Menne J, Buxhofer-Ausch V, Miesbach W
J Thromb Haemost 2020 Nov;18(11):3061-3066. Epub 2020 Sep 6 doi: 10.1111/jth.15045. PMID: 32757435Free PMC Article
Sargentini-Maier ML, De Decker P, Tersteeg C, Canvin J, Callewaert F, De Winter H
Expert Rev Clin Pharmacol 2019 Jun;12(6):537-545. Epub 2019 Apr 28 doi: 10.1080/17512433.2019.1607293. PMID: 30977686
Eskazan AE
Ann Hematol 2016 Oct;95(11):1751-6. Epub 2016 Sep 3 doi: 10.1007/s00277-016-2804-x. PMID: 27590601

Clinical prediction guides

Español I, Leal JD, Blanquer M, García-Candel F, Heredia A, Gómez-Espuch J, González C, Montserrat J, Díaz-Carrasco MS, Martínez A, Moraleda JM
Ann Hematol 2023 Aug;102(8):2069-2075. Epub 2023 May 12 doi: 10.1007/s00277-023-05248-9. PMID: 37171598Free PMC Article
Del Río-Garma J, Bobillo S, de la Rubia J, Pascual C, García-Candel F, García-Gala JM, Gonzalez R, Abril L, Vidan J, Gomez MJ, Peña F, Arbona C, Martín-Sanchez J, Moreno G, Romón I, Viejo A, Oliva A, Linares M, Salinas R, Pérez S, Garcia-Erce JA, Pereira A; “Registro Español de la Púrpura Trombocitopénica Trombótica (REPTT)”, and the “Grupo Español de Aféresis (GEA)”
Ann Hematol 2022 Jan;101(1):59-67. Epub 2021 Oct 12 doi: 10.1007/s00277-021-04685-8. PMID: 34642787
Völker LA, Brinkkoetter PT, Knöbl PN, Krstic M, Kaufeld J, Menne J, Buxhofer-Ausch V, Miesbach W
J Thromb Haemost 2020 Nov;18(11):3061-3066. Epub 2020 Sep 6 doi: 10.1111/jth.15045. PMID: 32757435Free PMC Article
Agosti P, Mancini I, Artoni A, Ferrari B, Pontiggia S, Trisolini SM, Facchini L, Peyvandi F; Italian Group of TTP Investigators
Thromb Res 2020 Mar;187:197-201. Epub 2019 Oct 22 doi: 10.1016/j.thromres.2019.10.010. PMID: 31685248
Sargentini-Maier ML, De Decker P, Tersteeg C, Canvin J, Callewaert F, De Winter H
Expert Rev Clin Pharmacol 2019 Jun;12(6):537-545. Epub 2019 Apr 28 doi: 10.1080/17512433.2019.1607293. PMID: 30977686

Recent systematic reviews

Soto-Mora JA, Gómez-Espitia LM, Lasalvia P, Castellanos Moreno CA, Casallas Vanegas CA, Londoño Gutiérrez SA
Int J Technol Assess Health Care 2023 Jul 21;39(1):e48. doi: 10.1017/S0266462323000442. PMID: 37476982Free PMC Article
Miesbach W, Menne J, Bommer M, Schönermarck U, Feldkamp T, Nitschke M, Westhoff TH, Seibert FS, Woitas R, Sousa R, Wolf M, Walzer S, Schwander B
Orphanet J Rare Dis 2019 Nov 15;14(1):260. doi: 10.1186/s13023-019-1240-0. PMID: 31730475Free PMC Article
Owattanapanich W, Wongprasert C, Rotchanapanya W, Owattanapanich N, Ruchutrakool T
Clin Appl Thromb Hemost 2019 Jan-Dec;25:1076029618825309. doi: 10.1177/1076029618825309. PMID: 30808221Free PMC Article

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