Glanzmann Thrombasthenia Medication
- Author: Zonera Ashraf Ali, MBBS; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP more...
The goals of pharmacotherapy in patients with Glanzmann thrombasthenia are to induce active immunity, reduce morbidity, and prevent complications. Platelet transfusions are the principal form of therapy, but recombinant activated factor VII (rFVIIa) can be used in patients with antibodies that render transfusions ineffective. Vaccination against hepatitis B is indicated, because of the increased risk of infection with multiple transfusions. In women, oral contraceptives may be used to control menorrhagia.
Coagulation Factor VIIa
Coagulation factor agents promote hemostasis.
Vitamin K–dependent glycoprotein that promotes hemostasis by activating the extrinsic pathway of the coagulation cascade, forming complexes with tissue factor. Promotes activation of factor X to factor Xa, factor IX to factor IXa, and factor II to factor IIa. It is indicated for treatment of bleeding episodes and perioperative management in adults and children with Glanzmann thrombasthenia with refractoriness to platelet transfusions, with or without antibodies to platelets.
Estrogen-progestin combinations of oral contraceptives reduce the secretion of LH and FSH from the pituitary by decreasing amount of gonadotropin-releasing hormones (GnRHs).
Suggested mechanisms by which hormonal therapy might affect bleeding include improvement in coagulation, alterations in the microvascular circulation, and improvements in endothelial integrity. One active tablet contains ethinyl estradiol 0.05 mg and norethindrone 1 mg. Used in women to prevent menorrhagia.
Malik U, Dutcher JP, Oleksowicz L. Acquired Glanzmann's thrombasthenia associated with Hodgkin's lymphoma: a case report and review of the literature. Cancer. 1998 May 1. 82(9):1764-8. [Medline]. [Full Text].
Poon MC, Zotz R, Di Minno G, et al. Glanzmann's thrombasthenia treatment: a prospective observational registry on the use of recombinant human activated factor VII and other hemostatic agents. Semin Hematol. 2006 Jan. 43(1 suppl 1):S33-6. [Medline].
Poon MC. Clinical use of recombinant human activated factor VII (rFVIIa) in the prevention and treatment of bleeding episodes in patients with Glanzmann's thrombasthenia. Vasc Health Risk Manag. 2007. 3(5):655-64. [Medline]. [Full Text].
Saxena R, Kannan M. Glanzmann's thrombasthenia: an overview. Clin Appl Thromb Hemost. 2008 Oct 16. epub ahead of print. [Medline].
van de Vijver E, De Cuyper IM, Gerrits AJ, Verhoeven AJ, Seeger K, Gutiérrez L, et al. Defects in Glanzmann thrombasthenia and LAD-III (LAD-1/v) syndrome: the role of integrin ß1 and ß3 in platelet adhesion to collagen. Blood. 2011 Nov 7. [Medline].
Nurden AT, Fiore M, Nurden P, Pillois X. Glanzmann thrombasthenia: a review of ITGA2B and ITGB3 defects with emphasis on variants, phenotypic variability, and mouse models. Blood. 2011 Dec 1. 118(23):5996-6005. [Medline].
Fiore M, Pillois X, Nurden P, Nurden AT, Austerlitz F. Founder effect and estimation of the age of the French Gypsy mutation associated with Glanzmann thrombasthenia in Manouche families. Eur J Hum Genet. 2011 Sep. 19(9):981-7. [Medline]. [Full Text].
Siddiq S, Clark A, Mumford A. A systematic review of the management and outcomes of pregnancy in Glanzmann thrombasthenia. Haemophilia. 2011 Sep. 17(5):e858-69. [Medline].
Tholouli E, Hay CR, O'Gorman P, Makris M. Acquired Glanzmann's thrombasthenia without thrombocytopenia: a severe acquired autoimmune bleeding disorder. Br J Haematol. 2004 Oct. 127(2):209-13. [Medline].
Kannan M, Chatterjee T, Ahmad F, Kumar R, Choudhry VP, Saxena R. Acquired Glanzmann's thrombasthenia associated with Hairy cell leukaemia. Eur J Clin Invest. 2009 Dec. 39(12):1110-1. [Medline].
Rajpurkar M, Chitlur M, Recht M, Cooper DL. Use of recombinant activated factor VII in patients with Glanzmann's thrombasthenia: a review of the literature. Haemophilia. 2014 Jul. 20(4):464-71. [Medline].
Ali N, Moiz B, Shaikh U, et al. Diagnostic tool for Glanzmann's thrombasthenia clinicopathologic spectrum. J Coll Physicians Surg Pak. 2008 Feb. 18(2):91-4. [Medline].
Jin PP, Shen WZ, Yang F, et al. [Analysis of clinical features and genotype in three Chinese pedigrees with Glanzmann thrombasthenia]. Zhonghua Xue Ye Xue Za Zhi. 2008 Mar. 29(3):149-53. [Medline].
Lombardo VT, Sottilotta G. Recombinant activated factor VII combined with desmopressin in preventing bleeding from dental extraction in a patient with Glanzmann's thrombasthenia. Clin Appl Thromb Hemost. 2006 Jan. 12(1):115-6. [Medline].
Shen WZ, Ding QL, Jin PP, et al. A novel Pro126His beta propeller mutation in integrin alphaIIb causes Glanzmann thrombasthenia by impairing progression of pro-alphaIIbbeta3 from endoplasmic reticulum to Golgi. Blood Cells Mol Dis. 2008 Oct 29. epub ahead of print. [Medline].
Solh M, Mescher C, Klappa A, Shah S, Zantek N, Datta Y. Acquired Glanzmann's thrombasthenia with optimal response to rituximab therapy. Am J Hematol. 2011 Aug. 86(8):715-6. [Medline].
Vuckovic SA. Glanzmann's thrombasthenia revisited. J Emerg Med. 1996 May-Jun. 14(3):299-303. [Medline].