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Impaired thrombin-induced platelet aggregation

MedGen UID:
868748
Concept ID:
C4023153
Finding
HPO: HP:0011872

Definition

Abnormal response to thrombin or thrombin mimetics as manifested by reduced or lacking aggregation of platelets upon addition of thrombin (or thrombin mimetics). [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Impaired thrombin-induced platelet aggregation

Conditions with this feature

Gray platelet syndrome
MedGen UID:
82900
Concept ID:
C0272302
Disease or Syndrome
The gray platelet syndrome (GPS) is a rare inherited disorder characterized by mild to moderate bleeding tendency, moderate thrombocytopenia, and a marked decrease or absence of platelet alpha-granules and of the proteins contained in alpha-granules. The platelets are enlarged, but not giant, and have a gray appearance on light microscopy of Wright-stained peripheral blood smears due to decreased granules. Many patients with gray platelet syndrome develop a stable myelofibrosis (summary by Nurden and Nurden, 2007). Cases suggesting autosomal dominant and autosomal recessive inheritance have been described, indicating that GPS is probably a genetically heterogeneous disorder with more than one molecular cause.

Professional guidelines

PubMed

Watanabe M, Siddiqui FM, Qureshi AI
Neurocrit Care 2012 Feb;16(1):203-9. doi: 10.1007/s12028-011-9591-y. PMID: 21748501

Recent clinical studies

Etiology

Murase M, Nakayama Y, Sessler DI, Mukai N, Ogawa S, Nakajima Y
Br J Anaesth 2017 Dec 1;119(6):1118-1126. doi: 10.1093/bja/aex349. PMID: 29040496
Watanabe M, Siddiqui FM, Qureshi AI
Neurocrit Care 2012 Feb;16(1):203-9. doi: 10.1007/s12028-011-9591-y. PMID: 21748501
Coccheri S
Drugs 2010 May 7;70(7):887-908. doi: 10.2165/11536000-000000000-00000. PMID: 20426498
Rosado JA, Nuñez AM, Lopez JJ, Pariente JA, Salido GM
Arch Biochem Biophys 2006 Aug 1;452(1):9-16. Epub 2006 Jun 16 doi: 10.1016/j.abb.2006.05.011. PMID: 16824477
Abbott L, Nadler J, Rude RK
Alcohol Clin Exp Res 1994 Oct;18(5):1076-82. doi: 10.1111/j.1530-0277.1994.tb00084.x. PMID: 7847587

Diagnosis

French SL, Thalmann C, Bray PF, Macdonald LE, Murphy AJ, Sleeman MW, Hamilton JR
Blood Adv 2018 Jun 12;2(11):1283-1293. doi: 10.1182/bloodadvances.2017015552. PMID: 29884748Free PMC Article
Kaur H, Corscadden K, Ware J, Othman M
Thromb Haemost 2017 Feb 28;117(3):543-555. Epub 2016 Dec 22 doi: 10.1160/TH16-04-0317. PMID: 28004055
Larocca LM, Heller PG, Podda G, Pujol-Moix N, Glembotsky AC, Pecci A, Alberelli MA, Balduini CL, Landolfi R, Cattaneo M, De Candia E
Platelets 2015;26(8):751-7. Epub 2015 Mar 25 doi: 10.3109/09537104.2014.994093. PMID: 25806575
Watanabe M, Siddiqui FM, Qureshi AI
Neurocrit Care 2012 Feb;16(1):203-9. doi: 10.1007/s12028-011-9591-y. PMID: 21748501
Cattaneo M, Canciani MT, Lecchi A, Kinlough-Rathbone RL, Packham MA, Mannucci PM, Mustard JF
Blood 1990 Mar 1;75(5):1081-6. PMID: 2137716

Therapy

French SL, Thalmann C, Bray PF, Macdonald LE, Murphy AJ, Sleeman MW, Hamilton JR
Blood Adv 2018 Jun 12;2(11):1283-1293. doi: 10.1182/bloodadvances.2017015552. PMID: 29884748Free PMC Article
Watanabe M, Siddiqui FM, Qureshi AI
Neurocrit Care 2012 Feb;16(1):203-9. doi: 10.1007/s12028-011-9591-y. PMID: 21748501
Coccheri S
Drugs 2010 May 7;70(7):887-908. doi: 10.2165/11536000-000000000-00000. PMID: 20426498
Galán C, Zbidi H, Bartegi A, Salido GM, Rosado JA
Arch Biochem Biophys 2009 Oct 15;490(2):137-44. Epub 2009 Aug 18 doi: 10.1016/j.abb.2009.08.007. PMID: 19695217
Sakurai Y, Shima M, Giddings J, Takeyama M, Kasuda S, Nogami K, Nishiya K, Yoshioka A
Thromb Res 2004;113(5):311-8. doi: 10.1016/j.thromres.2004.03.015. PMID: 15183043

Prognosis

Patel YM, Lordkipanidzé M, Lowe GC, Nisar SP, Garner K, Stockley J, Daly ME, Mitchell M, Watson SP, Austin SK, Mundell SJ
J Thromb Haemost 2014 May;12(5):716-25. doi: 10.1111/jth.12539. PMID: 24612435
Watanabe M, Siddiqui FM, Qureshi AI
Neurocrit Care 2012 Feb;16(1):203-9. doi: 10.1007/s12028-011-9591-y. PMID: 21748501
Rendu F, Marche P, Hovig T, Maclouf J, Lebret M, Tenza D, Levy-Toledano S, Caen JP
Br J Haematol 1987 Oct;67(2):199-206. doi: 10.1111/j.1365-2141.1987.tb02327.x. PMID: 2823861

Clinical prediction guides

Lopez-Vilchez I, Jerez-Dolz D, Diaz-Ricart M, Navarro V, Zafar MU, Zamorano-Leon J, Lopez-Farre A, Badimon JJ, Gasto C, Escolar G
Thromb Haemost 2017 Dec;117(12):2312-2321. Epub 2017 Dec 6 doi: 10.1160/TH17-04-0288. PMID: 29212119
Murase M, Nakayama Y, Sessler DI, Mukai N, Ogawa S, Nakajima Y
Br J Anaesth 2017 Dec 1;119(6):1118-1126. doi: 10.1093/bja/aex349. PMID: 29040496
Kaur H, Corscadden K, Ware J, Othman M
Thromb Haemost 2017 Feb 28;117(3):543-555. Epub 2016 Dec 22 doi: 10.1160/TH16-04-0317. PMID: 28004055
Larocca LM, Heller PG, Podda G, Pujol-Moix N, Glembotsky AC, Pecci A, Alberelli MA, Balduini CL, Landolfi R, Cattaneo M, De Candia E
Platelets 2015;26(8):751-7. Epub 2015 Mar 25 doi: 10.3109/09537104.2014.994093. PMID: 25806575
Pogliani EM, Fantasia R, Lambertenghi-Deliliers G, Cofrancesco E
Thromb Haemost 1981 Feb 23;45(1):38-42. PMID: 7245125

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