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Abnormal platelet phosphatidylserine exposure

MedGen UID:
1386143
Concept ID:
C4476990
Finding
Synonym: Abnormal plalelet phosphatidylserine exposure
 
HPO: HP:0031131

Definition

An abnormality of phosphatidylserine (PS) on activated platelets. PS is normally located on the cytoplasmic face of the resting platelet membrane but appears on the plasma-oriented surface of discrete membrane vesicles that derive from activated platelets. Thrombin, the central molecule of coagulation, is produced from prothrombin by a complex (prothrombinase) between factor Xa and its protein cofactor (factor V(a)) that forms on platelet-derived membranes. This complex enhances the rate of activation of prothrombin to thrombin by roughly 150,000 fold relative to factor X(a) in solution. The negatively charged surface of PS-containing platelet-derived membranes is at least partly responsible for this rate enhancement. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAbnormal platelet phosphatidylserine exposure

Recent clinical studies

Etiology

Pavlou EG, Georgatzakou HT, Fortis SP, Tsante KA, Tsantes AG, Nomikou EG, Kapota AI, Petras DI, Venetikou MS, Papageorgiou EG, Antonelou MH, Kriebardis AG
Biomolecules 2021 Sep 4;11(9) doi: 10.3390/biom11091309. PMID: 34572522Free PMC Article
Whelihan MF, Lim MY, Mooberry MJ, Piegore MG, Ilich A, Wogu A, Cai J, Monroe DM, Ataga KI, Mann KG, Key NS
J Thromb Haemost 2016 Oct;14(10):1941-1952. Epub 2016 Aug 31 doi: 10.1111/jth.13416. PMID: 27430959
Setty BN, Rao AK, Stuart MJ
Blood 2001 Dec 1;98(12):3228-33. doi: 10.1182/blood.v98.12.3228. PMID: 11719358

Diagnosis

Heubel-Moenen FCJI, Brouns SLN, Herfs L, Boerenkamp LS, Jooss NJ, Wetzels RJH, Verhezen PWM, Machiels P, Megy K, Downes K, Heemskerk JWM, Beckers EAM, Henskens YMC
Br J Haematol 2022 Mar;196(6):1388-1400. Epub 2022 Jan 10 doi: 10.1111/bjh.18003. PMID: 35001370Free PMC Article
Shcherbina A, Rosen FS, Remold-O'Donnell E
Br J Haematol 1999 Sep;106(4):875-83. doi: 10.1046/j.1365-2141.1999.01637.x. PMID: 10519987

Therapy

Ding W, Kou J, Meng H, Kou Y, He Z, Cao M, Wang L, Bi Y, Thatte HS, Shi J
Thromb Res 2015 Aug;136(2):354-60. Epub 2015 Jun 12 doi: 10.1016/j.thromres.2015.06.015. PMID: 26099643
Ataga KI, Cappellini MD, Rachmilewitz EA
Br J Haematol 2007 Oct;139(1):3-13. doi: 10.1111/j.1365-2141.2007.06740.x. PMID: 17854302
Rinder HM, Snyder EL, Tracey JB, Dincecco D, Wang C, Baril L, Rinder CS, Smith BR
Transfusion 2003 Sep;43(9):1230-7. doi: 10.1046/j.1537-2995.2003.00484.x. PMID: 12919425

Clinical prediction guides

Heubel-Moenen FCJI, Brouns SLN, Herfs L, Boerenkamp LS, Jooss NJ, Wetzels RJH, Verhezen PWM, Machiels P, Megy K, Downes K, Heemskerk JWM, Beckers EAM, Henskens YMC
Br J Haematol 2022 Mar;196(6):1388-1400. Epub 2022 Jan 10 doi: 10.1111/bjh.18003. PMID: 35001370Free PMC Article
Baroni Pietto MC, Lev PR, Glembotsky AC, Marín Oyarzún CP, Gomez G, Collado V, Pisoni C, Gomez RA, Grodzielski M, Gonzalez J, Mariño KV, Heller PG, Goette NP, Marta RF
Platelets 2022 Jul 4;33(5):743-754. Epub 2021 Nov 21 doi: 10.1080/09537104.2021.1988547. PMID: 34806522
Whelihan MF, Lim MY, Mooberry MJ, Piegore MG, Ilich A, Wogu A, Cai J, Monroe DM, Ataga KI, Mann KG, Key NS
J Thromb Haemost 2016 Oct;14(10):1941-1952. Epub 2016 Aug 31 doi: 10.1111/jth.13416. PMID: 27430959
Ataga KI, Cappellini MD, Rachmilewitz EA
Br J Haematol 2007 Oct;139(1):3-13. doi: 10.1111/j.1365-2141.2007.06740.x. PMID: 17854302
Rinder HM, Snyder EL, Tracey JB, Dincecco D, Wang C, Baril L, Rinder CS, Smith BR
Transfusion 2003 Sep;43(9):1230-7. doi: 10.1046/j.1537-2995.2003.00484.x. PMID: 12919425

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