Glanzmann Thrombasthenia: A Case Report in Moroccan Child
American Journal of Laboratory Medicine
Volume 3, Issue 5, September 2018, Pages: 44-47
Received: Oct. 14, 2018;
Accepted: Nov. 1, 2018;
Published: Nov. 28, 2018
Views 654 Downloads 68
Zohair Ait Ouzdi, Department of Hematology, the Mohamed VI University Hospital, Marrakech, Morocco
Fatimazahra Marhoume, Department of Hematology, the Mohamed VI University Hospital, Marrakech, Morocco
Khalil Haouach, Department of Hematology, the Mohamed VI University Hospital, Marrakech, Morocco
Follow on us
Glanzmann thrombasthenia is a hereditary hemorrhagic problem and is characterized by a qualitative or quantitative deficiency of the platelet glycoprotein GPIIb-IIIa, responsible for the reduction of platelet aggregation. The reported case concerns an 11-year-old child admitted to the Clinical Hematology Department of the University Hospital Mohamed 6 for epistaxis and gingivorrhagia. The questioning of the father revealed the concept of consanguinity, as well as his history of death and his hemorrhagic syndrome. The biological assessment performed in a patient shows a normal blood count; it is not particularly thrombocytopenia and the platelets have an average volume of 10.6 fL, the prothrombin time (PT), the partial thromboplastin time with activator (APTT) are normal. The platelet occlusion time (POT) is lengthened. The von Willebrand factor has not been investigated: the antigen (VWF-Ag) is 148% and the cofactor of ristocetin (VWF-RCo) is 116%. On the other hand, the exploration of platelet functions shows a lack of aggregation or of the platelet activator used: there is no aggregation in the presence of collagen, ADP, acid arachidonic, and aggregation induced by ristocetin is very disturbed. Immunophenotyping of platelet glycoproteins and the study of glycoprotein expression after thrombin activation by flow cytometry resulting from GpIIbIIIa (CD41), GpIIIa (CD61). The diagnosis is available for this patient is Glanzmann trombasthenia.
Glanzmann Thrombasthenia, Hemorrhagic Syndrome, Deficiency of Glycoprotein, GP IIb-IIIa
To cite this article
Zohair Ait Ouzdi,
Glanzmann Thrombasthenia: A Case Report in Moroccan Child, American Journal of Laboratory Medicine.
Vol. 3, No. 5,
2018, pp. 44-47.
Copyright © 2018 Authors retain the copyright of this article.
This article is an open access article distributed under the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/
) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Gobbur RH, Varghese I. A rare case of Glanzmann Thrombasthenia. Int J Med Sci Public Health 2014; 3.
Nurden P, Nurden A. Pathologies plaquettaires héréditaires. In: Sampol J, Arnoux D, Boutière B, et al. Manuel d'hémostase. Paris: Éditionsscientifiques et médicales Elsevier, 1995: 277-309.
Malik U, Dutcher JP, Oleksowicz L. Acquired. Glanzmann'sthrombasthenia associated with Hodgkin's lymphoma: a case report and review of the literature. Cancer 1998; 82: 1764-8.
French DL. The molecular genetics of Glanzmann's thrombasthenia. Platelets 1998; 9: 5-20.
George JN, Caen JP, Nurden AT. Glanzmann’s thrombasthenia: the spectrum of clinical disease. Blood. 1990; 75: 1383-95.
Caen JP. Glanzmann's thrombasthenia. Baillieres Clin Haematol. 1989; 2: 609–625.
Caen JP. Glanzmann’s thrombasthenia. Clin Haematol. 1972; 1: 383-92.
Awidi AS. Increased incidence of Glanzmann’s thrombasthenia in Jordan compared with Scandinavia. Scand J Haematol. 1983; 30: 218-22.
Monrigal. C, Beurrier. P, Mercier. FJ, Boyer-Neumann. C, P. Gillard. Glanzmann’s Thrombasthenia and pregnancy: a case and review of the literature. Annales Françaises d’Anesthésie et de Réanimation 22 (2003) 826–830.
Stepanian. A et al. Diagnostic de la maladie de Glanzmann chez un patient de 66 ans. Annales de biologie cliniques (2001) 660-3.
Jean-Louis Ntumba Mukendi, Souad Benkirane, Azlarab Masrar. Thrombasthénie de Glanzmann: à propos de 11 cas. Pan African Medical Journal. 2015; 21: 268.
Christopher Sebastiano, Michael Bromberg, Karen Breen, Matthew T. Hurford. Glanzmann’s thrombasthenia: report of a case and review of the literature. Int J Clin Exp Pathol 2010; 3(4): 443-447.
Lecompte P. Exploration des fonctions plaquettaires en pratique clinique. Spectra Biologie 1999; 18: 21-6.
Abdul Khalid, Tariq Baqai, Murtaza Bukhari Glanzmann`s Thrombosthenia: A rare cause of Recurrent Epistaxis. Int. j. pathol 2013; 11(2): 78-81
Harrison P, Mackie I, Mumford A, Briggs C, Liesner R, Winter M et al. Guidelines for the laboratory investigation of heritable disorders of platelet function. Br J Haematol. 2011; 155(1): 30- 44.
Solh T, Botsford A, Solh M. Glanzmann’s thrombasthenia: Pathogenesis, diagnosis, and current and emerging treatment options. J Blood Med. 2015; 6: 219-7.