Glanzmann Thrombasthenia: A Case Report in Moroccan Child

Zohair Ait Ouzdi; Fatimazahra Marhoume; Khalil Haouach

doi:doi:10.11648/j.ajlm.20180305.12

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Glanzmann Thrombasthenia: A Case Report in Moroccan Child

Zohair Ait Ouzdi, Fatimazahra Marhoume, Khalil Haouach

Published in American Journal of Laboratory Medicine (Volume 3, Issue 5)

Received: 14 October 2018 Accepted: 1 November 2018 Published: 28 November 2018

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Abstract

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.

Published in	American Journal of Laboratory Medicine (Volume 3, Issue 5)
DOI	10.11648/j.ajlm.20180305.12
Page(s)	44-47
Creative Commons	This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.
Copyright	Copyright © The Author(s), 2018. Published by Science Publishing Group

Keywords

Glanzmann Thrombasthenia, Hemorrhagic Syndrome, Deficiency of Glycoprotein, GP IIb-IIIa

References

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[2]	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.
[3]	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.
[4]	French DL. The molecular genetics of Glanzmann's thrombasthenia. Platelets 1998; 9: 5-20.
[5]	George JN, Caen JP, Nurden AT. Glanzmann’s thrombasthenia: the spectrum of clinical disease. Blood. 1990; 75: 1383-95.
[6]	Caen JP. Glanzmann's thrombasthenia. Baillieres Clin Haematol. 1989; 2: 609–625.
[7]	Caen JP. Glanzmann’s thrombasthenia. Clin Haematol. 1972; 1: 383-92.
[8]	Awidi AS. Increased incidence of Glanzmann’s thrombasthenia in Jordan compared with Scandinavia. Scand J Haematol. 1983; 30: 218-22.
[9]	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.
[10]	Stepanian. A et al. Diagnostic de la maladie de Glanzmann chez un patient de 66 ans. Annales de biologie cliniques (2001) 660-3.
[11]	Jean-Louis Ntumba Mukendi, Souad Benkirane, Azlarab Masrar. Thrombasthénie de Glanzmann: à propos de 11 cas. Pan African Medical Journal. 2015; 21: 268.
[12]	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.
[13]	Lecompte P. Exploration des fonctions plaquettaires en pratique clinique. Spectra Biologie 1999; 18: 21-6.
[14]	Abdul Khalid, Tariq Baqai, Murtaza Bukhari Glanzmann`s Thrombosthenia: A rare cause of Recurrent Epistaxis. Int. j. pathol 2013; 11(2): 78-81
[15]	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.
[16]	Solh T, Botsford A, Solh M. Glanzmann’s thrombasthenia: Pathogenesis, diagnosis, and current and emerging treatment options. J Blood Med. 2015; 6: 219-7.

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APA Style

Zohair Ait Ouzdi, Fatimazahra Marhoume, Khalil Haouach. (2018). Glanzmann Thrombasthenia: A Case Report in Moroccan Child. American Journal of Laboratory Medicine, 3(5), 44-47. https://doi.org/10.11648/j.ajlm.20180305.12

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ACS Style

Zohair Ait Ouzdi; Fatimazahra Marhoume; Khalil Haouach. Glanzmann Thrombasthenia: A Case Report in Moroccan Child. Am. J. Lab. Med. 2018, 3(5), 44-47. doi: 10.11648/j.ajlm.20180305.12

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AMA Style

Zohair Ait Ouzdi, Fatimazahra Marhoume, Khalil Haouach. Glanzmann Thrombasthenia: A Case Report in Moroccan Child. Am J Lab Med. 2018;3(5):44-47. doi: 10.11648/j.ajlm.20180305.12

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@article{10.11648/j.ajlm.20180305.12,
  author = {Zohair Ait Ouzdi and Fatimazahra Marhoume and Khalil Haouach},
  title = {Glanzmann Thrombasthenia: A Case Report in Moroccan Child},
  journal = {American Journal of Laboratory Medicine},
  volume = {3},
  number = {5},
  pages = {44-47},
  doi = {10.11648/j.ajlm.20180305.12},
  url = {https://doi.org/10.11648/j.ajlm.20180305.12},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajlm.20180305.12},
  abstract = {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.},
 year = {2018}
}

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TY  - JOUR
T1  - Glanzmann Thrombasthenia: A Case Report in Moroccan Child
AU  - Zohair Ait Ouzdi
AU  - Fatimazahra Marhoume
AU  - Khalil Haouach
Y1  - 2018/11/28
PY  - 2018
N1  - https://doi.org/10.11648/j.ajlm.20180305.12
DO  - 10.11648/j.ajlm.20180305.12
T2  - American Journal of Laboratory Medicine
JF  - American Journal of Laboratory Medicine
JO  - American Journal of Laboratory Medicine
SP  - 44
EP  - 47
PB  - Science Publishing Group
SN  - 2575-386X
UR  - https://doi.org/10.11648/j.ajlm.20180305.12
AB  - 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.
VL  - 3
IS  - 5
ER  -

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Author Information

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

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APA Style

Zohair Ait Ouzdi, Fatimazahra Marhoume, Khalil Haouach. (2018). Glanzmann Thrombasthenia: A Case Report in Moroccan Child. American Journal of Laboratory Medicine, 3(5), 44-47. https://doi.org/10.11648/j.ajlm.20180305.12

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ACS Style

Zohair Ait Ouzdi; Fatimazahra Marhoume; Khalil Haouach. Glanzmann Thrombasthenia: A Case Report in Moroccan Child. Am. J. Lab. Med. 2018, 3(5), 44-47. doi: 10.11648/j.ajlm.20180305.12

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AMA Style

Zohair Ait Ouzdi, Fatimazahra Marhoume, Khalil Haouach. Glanzmann Thrombasthenia: A Case Report in Moroccan Child. Am J Lab Med. 2018;3(5):44-47. doi: 10.11648/j.ajlm.20180305.12

Copy | Download

@article{10.11648/j.ajlm.20180305.12,
  author = {Zohair Ait Ouzdi and Fatimazahra Marhoume and Khalil Haouach},
  title = {Glanzmann Thrombasthenia: A Case Report in Moroccan Child},
  journal = {American Journal of Laboratory Medicine},
  volume = {3},
  number = {5},
  pages = {44-47},
  doi = {10.11648/j.ajlm.20180305.12},
  url = {https://doi.org/10.11648/j.ajlm.20180305.12},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajlm.20180305.12},
  abstract = {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.},
 year = {2018}
}

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TY  - JOUR
T1  - Glanzmann Thrombasthenia: A Case Report in Moroccan Child
AU  - Zohair Ait Ouzdi
AU  - Fatimazahra Marhoume
AU  - Khalil Haouach
Y1  - 2018/11/28
PY  - 2018
N1  - https://doi.org/10.11648/j.ajlm.20180305.12
DO  - 10.11648/j.ajlm.20180305.12
T2  - American Journal of Laboratory Medicine
JF  - American Journal of Laboratory Medicine
JO  - American Journal of Laboratory Medicine
SP  - 44
EP  - 47
PB  - Science Publishing Group
SN  - 2575-386X
UR  - https://doi.org/10.11648/j.ajlm.20180305.12
AB  - 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.
VL  - 3
IS  - 5
ER  -

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