[ La thrombasthénie de Glanzmann chez l’enfant ]
Volume 33, Issue 2, July 2021, Pages 326–329
Sanaa Bouramdane1, Sarra Benmiloud2, Mohamed Hbibi3, and Moustapha Hida4
1 Unité d’hémato-oncologie pédiatrique, Service de pédiatrie, Hôpital Mère-Enfant, CHU Hassan II, Fès, Morocco
2 Faculté de Médecine et de Pharmacie de Fès, Université Sidi Mohamed Ben Abdellah, Maroc
3 Faculté de Médecine et de Pharmacie de Fès, Université Sidi Mohamed Ben Abdellah, Maroc
4 Service de pédiatrie et des urgences pédiatriques, Hôpital Mère - Enfant, CHU Hassan II, Fès, Morocco
Original language: French
Copyright © 2021 ISSR Journals. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Glanzmann thrombasthenia is a rare hereditary thrombopathy, mainly responsible for spontaneous mucocutaneous hemorrhages. It is due to a qualitative or quantitative deficiency of the platelet receptor GPIIbIIIa, involved in platelet aggregation. This disease is mostly observed in populations with high consanguinity. The aim of our work is to study the epidemiological, clinical, biological, therapeutic and evolutionary particularities of Glanzmann thrombasthenia in children. A total of 11 patients were diagnosed and followed up in the pediatric hematology-oncology department of the Hassan II University Hospital of Fez, Morocco. The average age at diagnosis was 2 years and 6 months, 81% of the cases were from consanguineous parents, mucocutaneous hemorrhages were the most reported, and the diagnosis of Glanzmann thrombasthenia was confirmed in all cases by aggregometry. Treatment is based mainly on local symptomatic measures and platelet transfusion, activated factor VII is reserved in case of anti-platelet immunization. The evolution is good in all cases, only one death is reported.
Author Keywords: Glanzmann thrombasthenia, thrombopathy, children, platelet, hemorrhage.
Volume 33, Issue 2, July 2021, Pages 326–329
Sanaa Bouramdane1, Sarra Benmiloud2, Mohamed Hbibi3, and Moustapha Hida4
1 Unité d’hémato-oncologie pédiatrique, Service de pédiatrie, Hôpital Mère-Enfant, CHU Hassan II, Fès, Morocco
2 Faculté de Médecine et de Pharmacie de Fès, Université Sidi Mohamed Ben Abdellah, Maroc
3 Faculté de Médecine et de Pharmacie de Fès, Université Sidi Mohamed Ben Abdellah, Maroc
4 Service de pédiatrie et des urgences pédiatriques, Hôpital Mère - Enfant, CHU Hassan II, Fès, Morocco
Original language: French
Copyright © 2021 ISSR Journals. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Glanzmann thrombasthenia is a rare hereditary thrombopathy, mainly responsible for spontaneous mucocutaneous hemorrhages. It is due to a qualitative or quantitative deficiency of the platelet receptor GPIIbIIIa, involved in platelet aggregation. This disease is mostly observed in populations with high consanguinity. The aim of our work is to study the epidemiological, clinical, biological, therapeutic and evolutionary particularities of Glanzmann thrombasthenia in children. A total of 11 patients were diagnosed and followed up in the pediatric hematology-oncology department of the Hassan II University Hospital of Fez, Morocco. The average age at diagnosis was 2 years and 6 months, 81% of the cases were from consanguineous parents, mucocutaneous hemorrhages were the most reported, and the diagnosis of Glanzmann thrombasthenia was confirmed in all cases by aggregometry. Treatment is based mainly on local symptomatic measures and platelet transfusion, activated factor VII is reserved in case of anti-platelet immunization. The evolution is good in all cases, only one death is reported.
Author Keywords: Glanzmann thrombasthenia, thrombopathy, children, platelet, hemorrhage.
Abstract: (french)
La thrombasthénie de Glanzmann est une thrombopathie héréditaire rare, responsable essentiellement d’hémorragies cutanéo-muqueuses spontanées. Elle est due à un déficit qualitatif ou quantitatif du récepteur plaquettaire GPIIbIIIa, impliqué dans l’agrégation des plaquettes. Cette maladie est surtout observée dans les populations à forte consanguinité. L’objectif de notre travail est d’étudier les particularités épidémiologiques, cliniques, biologiques, thérapeutiques et évolutives de la thrombasthénie de Glanzmann chez l’enfant. Au total, 11 patients sont diagnostiqués et suivis à l’unité d’hémato-oncologie pédiatrique du centre hospitalier universitaire Hassan II de Fès, Maroc. L’âge moyen au diagnostic est de 2 ans et 6 mois, 81% des cas sont issus de parents consanguins, les hémorragies cutanéo-muqueuses sont les plus rapportées, l’agrégométrie a permis de retenir le diagnostic de thrombasthénie de Glanzmann dans tous les cas. Le traitement est basé surtout sur les mesures symptomatiques locales et la transfusion plaquettaire, le facteur VII activé est réservé en cas d’immunisation antiplaquettaire. L’évolution est bonne pour tous les cas, un seul décès est rapporté.
Author Keywords: Thrombasthénie de Glanzmann, thrombopathie, enfant, plaquette, hémorragie.
How to Cite this Article
Sanaa Bouramdane, Sarra Benmiloud, Mohamed Hbibi, and Moustapha Hida, “Glanzmann thrombasthenia in children,” International Journal of Innovation and Applied Studies, vol. 33, no. 2, pp. 326–329, July 2021.
