Point-of-care platelet function testing results in a dog with Bernard-Soulier syndrome.
Plateletworks
macrothrombocytopathia
platelet function analyzer
Journal
Veterinary clinical pathology
ISSN: 1939-165X
Titre abrégé: Vet Clin Pathol
Pays: United States
ID NLM: 9880575
Informations de publication
Date de publication:
12 Jul 2023
12 Jul 2023
Historique:
revised:
05
04
2023
received:
29
12
2022
accepted:
24
04
2023
medline:
13
7
2023
pubmed:
13
7
2023
entrez:
12
7
2023
Statut:
aheadofprint
Résumé
Bernard-Soulier syndrome (BSS), also known as hemorrhagiparous thrombocytic dystrophy (OMIA 002207-9615), is a rare defect in platelet function recognized in both dogs and humans. It is caused by a deficiency in glycoprotein 1b-IX-V, the platelet surface protein which acts as a receptor for the von Willebrand factor. The characteristic features of BSS in humans and dogs include macrothrombocytes and mild-to-moderate thrombocytopenia with a bleeding tendency. This condition has previously been reported in European Cocker Spaniel dogs; however, the results of platelet function tests in these animals have not been reported. This case report describes a European Cocker Spaniel dog with spontaneously occurring Bernard-Soulier syndrome and the results of point-of-care platelet function tests, including a prolonged buccal mucosal bleeding time (>8 min), prolongation (>300 s) of PFA-200 COL/ADP, COL/EPI, and P2Y closure times, and reduced aggregation (15%-48%) with Plateletworks ADP, but with normal aggregation (92%) with Plateletworks AA. This is the first description of the results of platelet function tests in canine Bernard-Soulier syndrome.
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Subventions
Organisme : OVC Pet Trust
Informations de copyright
© 2023 The Authors. Veterinary Clinical Pathology published by Wiley Periodicals LLC on behalf of the American Society for Veterinary Clinical Pathology.
Références
Garden OA, Kidd L, Mexas AM, et al. ACVIM consensus statement on the diagnosis of immune-mediated hemolytic anemia in dogs and cats. J Vet Intern Med. 2019;33(2):313-334. doi:10.1111/JVIM.15441
Berndt MC, Andrews RK. Bernard-Soulier syndrome. Haematologica. 2011;96(3):355-359. doi:10.3324/haematol.2010.039883
Lanza F. Bernard-Soulier syndrome (hemorrhagiparous thrombocytic dystrophy). Orphanet J Rare Dis. 2006;1:46. doi:10.1186/1750-1172-1-46
Bragadottir G, Birgisdottir ER, Gudmundsdottir BR, et al. Clinical phenotype in heterozygote and biallelic Bernard-Soulier syndrome-a case control study. Am J Hematol. 2015;90(2):149-155. doi:10.1002/AJH.23891
Monteiro M, Almeida L, Morais M, Dias L. Bernard Soulier syndrome: a rare, frequently misdiagnosed and poorly managed bleeding disorder. BMJ Case Rep. 2021;14(8):e243518. doi:10.1136/BCR-2021-243518
Grainger JD, Thachil J, Will AM. How we treat the platelet glycoprotein defects; Glanzmann thrombasthenia and Bernard Soulier syndrome in children and adults. Br J Haematol. 2018;182(5):621-632. doi:10.1111/BJH.15409
Gentilini F, Turba ME, Giancola F, et al. A large deletion in the GP9 gene in cocker spaniel dogs with Bernard-Soulier syndrome. PloS One. 2019;14(9):e0220625. doi:10.1371/JOURNAL.PONE.0220625
Kunishima S, Kamiya T, Saito H. Genetic abnormalities of Bernard-Soulier syndrome. Int J Hematol. 2002;76(4):319-327. doi:10.1007/BF02982690
Callan MB, Bennett JS, Phillips DK, et al. Inherited platelet delta-storage pool disease in dogs causing severe bleeding: an animal model for a specific ADP deficiency. Thromb Haemost. 1995;74(3):949-953. doi:10.1055/s-0038-1649853
Christopherson PW, Spangler EA, Boudreaux MK. Evaluation and clinical application of platelet function testing in small animal practice. Vet Clinics North Am Small Anim Pract. 2012;42(1):173-188. doi:10.1016/j.cvsm.2011.09.013
Aumann M, Rossi V, le Boedec K, Diquélou A. Comparison of the buccal mucosal bleeding time in dogs using 3 different-sized lancet devices. Vet Clin Pathol. 2013;42(4):451-457. doi:10.1111/VCP.12087
Posan E, McBane R, Grill D, Motsko CL, Nichols WL. Comparison of PFA-100 testing and bleeding time for detecting platelet hypofunction and von Willebrand disease in clinical practice. Thromb Haemost. 2003;90(3):476-482. doi:10.1160/TH03-02-0111
Saati S, Abrams-Ogg ACG, Blois SL, Wood RD. Comparison of multiplate, platelet function Analyzer-200, and Plateletworks in healthy dogs treated with aspirin and clopidogrel. J Vet Intern Med. 2018;32(1):111-118. doi:10.1111/jvim.14886
Westgarth S, Blois SL, Wood RD, et al. Effects of omega-3 polyunsaturated fatty acids and aspirin, alone and combined, on canine platelet function. J Small Anim Pract. 2018 May;59(5):272-280. doi:10.1111/jsap.12776
Dickinson M, Abrams-Ogg A, Blois S, Wood RD, Monteith G. Extended sample storage for platelet function testing in healthy dogs. Vet Clin Pathol. 2023. Epub ahead of print. doi:10.1111/vcp.13226
Harrison P, Robinson MSC, Mackie IJ, et al. Performance of the platelet function analyser PFA-100 in testing abnormalities of primary haemostasis. Blood Coagul Fibrinolysis. 1999;10(1):25-31. doi:10.1097/00001721-199901000-00004
Tarnow I, Kristensen AT, Texel H, Olsen LH, Pedersen HD. Decreased platelet function in cavalier king Charles spaniels with mitral valve regurgitation. J Vet Intern Med. 2003;17(5):680-686. doi:10.1111/J.1939-1676.2003.TB02500.X
Nielsen LA, Zois NE, Pedersen HD, et al. Platelet function in dogs: breed differences and effect of acetylsalicylic acid administration. Vet Clin Pathol. 2007;36(3):267-273. doi:10.1111/J.1939-165X.2007.TB00222.X
Favaloro EJ, Bonar R. External quality assessment/proficiency testing and internal quality control for the PFA-100 and PFA-200: an update. Semin Thromb Hemost. 2014;40(2):239-253. doi:10.1055/S-0034-1365844/ID/JR02049-29
Lippi G, Fontana R, Avanzini P, et al. Influence of mechanical trauma of blood and hemolysis on PFA-100 testing. Blood Coagul Fibrinolysis. 2012;23(1):82-86. doi:10.1097/MBC.0B013E32834C6CB5
Alamelu J, Liesner R. Modern management of severe platelet function disorders. Br J Haematol. 2010;149(6):813-823. doi:10.1111/J.1365-2141.2010.08191.X
Favaloro EJ. The utility of the PFA-100 in the identification of von Willebrand disease: a concise review. Semin Thromb Hemost. 2006;32(5):537-545. doi:10.1055/S-2006-947869
Brooks MB, Randolph J, Warner K, Center S. Evaluation of platelet function screening tests to detect platelet procoagulant deficiency in dogs with Scott syndrome. Vet Clin Pathol. 2009;38(3):306-315. doi:10.1111/J.1939-165X.2009.00141.X
Paniccia R, Priora R, Liotta AA, Abbate R. Platelet function tests: a comparative review. Vasc Health Risk Manag. 2015;11:133-148. doi:10.2147/VHRM.S44469
Li RHL, Ontiveros E, Nguyen N, et al. Precision medicine identifies a pathogenic variant of the ITGA2B gene responsible for Glanzmann's thrombasthenia in a cat. J Vet Intern Med. 2020;34(6):2438-2446. doi:10.1111/jvim.15886doi:10.1111/jvim.15886
Burgess HJ, Woods JP, Abrams-Ogg AC, et al. Evaluation of laboratory methods to improve characterization of dogs with von Willebrand disease. Can Vet J. 2009;73(4):252-259.
Threlfall AJ, Boag AM, Soutter F, et al. Analysis of DLA-DQB1 and polymorphisms in CTLA4 in cocker spaniels affected with immune-mediated haemolytic anaemia. Canine Genet Epidemiol. 2015;2(1):1-10. doi:10.1186/S40575-015-0020-Y28
Prabu P, Parapia LA. Bernard-Soulier syndrome in pregnancy. Clin Lab Haematol. 2006;28(3):198-201. doi:10.1111/J.1365-2257.2006.00780.X