CONTROL OF SEVERE BLEEDING EPISODE IN CASE OF GLANZMANN'S THROMBASTHENIA REFRACTORY TO PLATELET TRANSFUSION THERAPY BY ADMINISTERING RECOMBINANT FACTOR VIIa
Abstract
lanzmann's thrombasthenia is an autosomal recessive inherited platelet function defect. Though,quantitatively normal, the aggregation ability of platelets is reduced leading to bleeding episodes
requiring transfusion of platelet concentrates. We describe a case of 13-year-old girl who had
recurrent episodes of epistaxis since birth and was managed with multiple platelet concentrate
transfusions and recently admitted with severe epistaxis refractory to platelet transfusion. At this
stage administration of recombinant activated factor VII (fVIIa) was considered, which was
initially given at 90 µg/kg dose with little control of bleeding but subsequent second dose of 120
µg/kg was administered with excellent response and immediate control of bleeding.
Keywords: Glanzmann's thrombasthenia, autosomal recessive, platelet disorder, recombinant
factor VIIa, epistaxis
References
Glanzmann E. Hereditare hamorrhagische thrombasthenie.
Ein Beitrag zur Pathologie der Blutplattchen. J
Kinderkranken 1918;88:113.
Wilcox DA, Wauthier JL, Pidard D, Newman PJ. A single
amino acid substitution flanking the fourth calcium binding
domain of αIIb prevents maturation of the αIIbβ3 complex. J
Biol Chem 1994;269:4450-7.
Ruiz C, Liu CY, Sun QH, Sigaud-Fiks M, Fressinaud E,
Muller JY, et al. A point mutation in the cysteine-rich
domain of glycoprotein (GP) IIIa results in the expression of
a GPIIb-IIIa (αIIbβ3) integrin receptor locked in a high
affinity state and a Glanzmann thrombasthenia-like
phenotype. Blood 2001;98:2432-41.
George JN, Caen JP, Nurden AT. Glanzmann's
thrombasthenia: The spectrum of clinical disease. Blood
;75:1383-95.
Nurden AT, George JN. Inherited abnormalities of the
platelet membrane: Glanzmann thrombasthenia, BernardSoulier syndrome, and other disorders. In: RW Colman, VJ
Marder, AW Clowes, JN George, SZ Goldhaber. (eds).
Hemostasis and Thrombosis, Basic Principles and Clinical
J Ayub Med Coll Abbottabad 2009;21(2)
http://www.ayubmed.edu.pk/JAMC/PAST/21-2/Asim.pdf 173
Practice. 4th edition. Philadelphia: Lippincott, Williams &
Wilkins; 2005. p.987-1010.
Bellucci S, Caen J. Molecular basis of Glanzmann's
thrombasthenia and current strategies in treatment. Blood
Rev 2002;16:193-202.
Martlew VJ. Peri-operative management of patients with
coagulation disorders. Br J Anaesth 2000;85:446-55.
Monte S, Lyons G. Peripartum management of a patient with
Glanzmann's thrombasthenia using Thrombelastograph. Br J
Anaesth 2001;87:734-8.
Martin I, Kriaa F, Proulle V, Guillet B, Kaplan C, D'Oiron R,
et al. Protein A Sepharose immunoadsorption can restore the
efficacy of platelet concentrates in most patients with
Glanzmann's thrombasthenia and anti-glycoprotein IIb-IIIa
antibodies.Br J Haematol 2002;119:991-7.
Ito K, Yoshida H, Hatoyama H, Matsumoto H, Ban C, Mori
T, et al. Antibody removal therapy used successfully at
delivery of a pregnant patient with Glanzmann's
thrombasthenia and multiple anti-platelet antibodies. Vox
Sang 1991;61:40-6.
Poon MC, D'Oiron R, Von Depka M, Khair K, Negrier C,
Karafoulidou A, et al. International Data Collection on
Recombinant Factor VIIa and Congenital Platelet Disorders
Study Group: Prophylactic and therapeutic recombinant
factor VIIa administration to patients with Glanzmann's
thrombasthenia: results of an international survey. J Thromb
Haemost 2004;2:1096-1103.
Bellucci S, Devergie A, Gluckman E, Tobelem G,
Lethielleux P, Benbunan M, et al. Complete correction of
Glanzmann's thrombasthenia by allogeneic bone-marrow
transplantation. Br J Haematol 1985;59:635-41.
Fujimoto TT, Kishimoto M. Glanzmann thrombasthenia with
acute myeloid leukemia successfully treated by bonemarrow
transplantation. Int J Hematol 2005;81(1):77-80.
Hoffman M. A cell-based model of hemostasis. Blood Rev
;17:S1-5.
Monroe DM, Hoffman M, Oliver JA, Roberts HR. Platelet
activity of high dose factor VIIa is independent of tissue
factor. Br J Haematol 1997;99:542-7.
Published
How to Cite
Issue
Section
License
Journal of Ayub Medical College, Abbottabad is an OPEN ACCESS JOURNAL which means that all content is FREELY available without charge to all users whether registered with the journal or not. The work published by J Ayub Med Coll Abbottabad is licensed and distributed under the creative commons License CC BY ND Attribution-NoDerivs. Material printed in this journal is OPEN to access, and are FREE for use in academic and research work with proper citation. J Ayub Med Coll Abbottabad accepts only original material for publication with the understanding that except for abstracts, no part of the data has been published or will be submitted for publication elsewhere before appearing in J Ayub Med Coll Abbottabad. The Editorial Board of J Ayub Med Coll Abbottabad makes every effort to ensure the accuracy and authenticity of material printed in J Ayub Med Coll Abbottabad. However, conclusions and statements expressed are views of the authors and do not reflect the opinion/policy of J Ayub Med Coll Abbottabad or the Editorial Board.
USERS are allowed to read, download, copy, distribute, print, search, or link to the full texts of the articles, or use them for any other lawful purpose, without asking prior permission from the publisher or the author. This is in accordance with the BOAI definition of open access.
AUTHORS retain the rights of free downloading/unlimited e-print of full text and sharing/disseminating the article without any restriction, by any means including twitter, scholarly collaboration networks such as ResearchGate, Academia.eu, and social media sites such as Twitter, LinkedIn, Google Scholar and any other professional or academic networking site.
