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New Insights Into the Treatment of Glanzmann Thrombasthenia.
Poon MC, Di Minno G, d'Oiron R, Zotz R. Poon MC, et al. Transfus Med Rev. 2016 Apr;30(2):92-9. doi: 10.1016/j.tmrv.2016.01.001. Epub 2016 Jan 30. Transfus Med Rev. 2016. PMID: 26968829 Free article. Review.
Glanzmann thrombasthenia (GT) is a rare inherited autosomal recessive bleeding disorder of platelet function caused by a quantitative or qualitative defect of platelet membrane glycoprotein IIb/IIIa (integrin alphaIIbbeta3), a fibrinogen receptor required for platel …
Glanzmann thrombasthenia (GT) is a rare inherited autosomal recessive bleeding disorder of platelet function caused by a quant …
Inherited platelet diseases with normal platelet count: phenotypes, genotypes and diagnostic strategy.
Nurden P, Stritt S, Favier R, Nurden AT. Nurden P, et al. Haematologica. 2021 Feb 1;106(2):337-350. doi: 10.3324/haematol.2020.248153. Haematologica. 2021. PMID: 33147934 Free PMC article. Review.
Inherited platelet disorders resulting from platelet function defects and a normal platelet count cause a moderate or severe bleeding diathesis. Since the description of Glanzmann thrombasthenia resulting from defects of ITGA2B and ITGB3, new inherited platelet diso …
Inherited platelet disorders resulting from platelet function defects and a normal platelet count cause a moderate or severe bleeding diathe …
Genetic testing in the diagnostic evaluation of inherited platelet disorders.
Nurden AT, Fiore M, Pillois X, Nurden P. Nurden AT, et al. Semin Thromb Hemost. 2009 Mar;35(2):204-12. doi: 10.1055/s-0029-1220328. Epub 2009 Apr 30. Semin Thromb Hemost. 2009. PMID: 19408193 Review.
But a full diagnosis can only be complete when the genetic defect has been defined for each patient. Glanzmann thrombasthenia (GT) and the Bernard-Soulier syndrome are the most studied of the membrane disorders and a wide range of mutations identified. ...Knowledge …
But a full diagnosis can only be complete when the genetic defect has been defined for each patient. Glanzmann thrombasthenia …
Anti-alpha(IIb) beta(3) immunization in Glanzmann thrombasthenia: review of literature and treatment recommendations.
Fiore M, d'Oiron R, Pillois X, Alessi MC. Fiore M, et al. Br J Haematol. 2018 Apr;181(2):173-182. doi: 10.1111/bjh.15087. Epub 2018 Apr 2. Br J Haematol. 2018. PMID: 29611179 Free article. Review.
Glanzmann thrombasthenia (GT) is caused by inherited defects of the alpha(II)(b) beta(3) platelet glycoprotein. This bleeding disorder can be treated with platelet transfusion therapy, but some patients will be immunized and begin to form anti-human leucocyte …
Glanzmann thrombasthenia (GT) is caused by inherited defects of the alpha(II)(b) beta(3) platelet glycoprotein. This bleeding …
The use of prophylaxis in the treatment of rare bleeding disorders.
Shapiro A. Shapiro A. Thromb Res. 2020 Dec;196:590-602. doi: 10.1016/j.thromres.2019.07.014. Epub 2019 Jul 19. Thromb Res. 2020. PMID: 31420204 Review.
Rare bleeding disorders (RBDs) are a heterogeneous group of coagulation factor deficiencies that include fibrinogen, prothrombin, alpha(2)-antiplasmin, plasminogen activator inhibitor-1, and factors II, V, V/VIII, VII, X, XI and XIII. ...Platelet disorders including Gla …
Rare bleeding disorders (RBDs) are a heterogeneous group of coagulation factor deficiencies that include fibrinogen, prothrombin, alpha(2 …
Molecular basis of Glanzmann's Thrombasthenia and current strategies in treatment.
Bellucci S, Caen J. Bellucci S, et al. Blood Rev. 2002 Sep;16(3):193-202. doi: 10.1016/s0268-960x(02)00030-9. Blood Rev. 2002. PMID: 12163005 Review.
Glanzmann Thrombasthenia, an exceptional inherited platelet disorder is characterized by a complete lack of platelet aggregation due to a defect in the alpha(IIb)beta(3) complex or to a qualitative abnormality of this complex. ...Hemorrhages are the main clinical pr …
Glanzmann Thrombasthenia, an exceptional inherited platelet disorder is characterized by a complete lack of platelet aggregati …
Iron deficiency anemia and bleeding management in pediatric patients with Bernard-Soulier syndrome and Glanzmann Thrombasthenia: A single-institution analysis.
Lee A, Maier CL, Batsuli G. Lee A, et al. Haemophilia. 2022 Jul;28(4):633-641. doi: 10.1111/hae.14559. Epub 2022 Apr 12. Haemophilia. 2022. PMID: 35412688 Free PMC article.
INTRODUCTION: Frequent and severe bleeding events (SBE) in patients with inherited qualitative platelet disorders Bernard-Soulier Syndrome (BSS) and Glanzmann Thrombasthenia (GT) can lead to secondary iron deficiency anemia (IDA). ...Following initiation of rFVIIa p …
INTRODUCTION: Frequent and severe bleeding events (SBE) in patients with inherited qualitative platelet disorders Bernard-Soulier Syndrome ( …
Clinical use of recombinant human activated factor VII (rFVIIa) in the prevention and treatment of bleeding episodes in patients with Glanzmann's thrombasthenia.
Poon MC. Poon MC. Vasc Health Risk Manag. 2007;3(5):655-64. Vasc Health Risk Manag. 2007. PMID: 18078017 Free PMC article. Review.
Glanzmann's thrombasthenia (GT) is a congenital qualitative platelet disorders due to the deficiency or defect of platelet membrane GPIIb/IIIa (integrin alpha(IIb)beta3). ...In the absence of this data, for treatment of severe bleeding in GT patients with platelet a …
Glanzmann's thrombasthenia (GT) is a congenital qualitative platelet disorders due to the deficiency or defect of platelet mem …
The evidence for the use of recombinant human activated factor VII in the treatment of bleeding patients with quantitative and qualitative platelet disorders.
Poon MC. Poon MC. Transfus Med Rev. 2007 Jul;21(3):223-36. doi: 10.1016/j.tmrv.2007.03.003. Transfus Med Rev. 2007. PMID: 17572261 Review.
In experimental models, this improved thrombin generation enhances platelet adhesion in thrombocytopenic conditions and enhances adhesion and aggregation of platelets lacking glycoprotein IIbIIIa (integrin alpha(IIb)beta(3)), characteristic of the qualitative platelet disorder …
In experimental models, this improved thrombin generation enhances platelet adhesion in thrombocytopenic conditions and enhances adhesion an …
Outpatient Management of Heavy Menstrual Bleeding in Adolescent and Young Women with Inherited Platelet Function Disorders.
Pennesi CM, Quint EH, Rosen MW, Compton SD, Odukoya EJ, Weyand AC. Pennesi CM, et al. J Pediatr Adolesc Gynecol. 2020 Oct;33(5):489-493. doi: 10.1016/j.jpag.2020.06.019. Epub 2020 Jun 28. J Pediatr Adolesc Gynecol. 2020. PMID: 32610148
After their initial visit, 19/34 (56%) were treated with HM, 12/34 (35%) with NHM, 2/34 (6%) with a combination of methods, and 1/34 (3%) were untreated. Initial treatment failed in 19/34 (56%) and those patients subsequently required a mean of 2 additional treatmen …
After their initial visit, 19/34 (56%) were treated with HM, 12/34 (35%) with NHM, 2/34 (6%) with a combination of methods, and 1/34 …
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