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Case Reports
. 2023 Mar 29:44:151.
doi: 10.11604/pamj.2023.44.151.38361. eCollection 2023.

[Scott's syndrome and pregnancy: about a case]

[Article in French]
Allae Eddine Bouchaib  1 , Mamadou Alpha Balde  1 , Abdellah Babahabib  1 , Moulay El Mehdi Elhassani  1 , Jaouad Kouach  1
Affiliations
Case Reports

[Scott's syndrome and pregnancy: about a case]

[Article in French]
Allae Eddine Bouchaib et al. Pan Afr Med J. .

Abstract
in English, French

Scott´s syndrome is a rare congenital thrombopathy with an autosomal recessive inheritance pattern. The purpose of our study is to report the case of a parturient woman with Scott´s syndrome secondary to a mutation of the ANO6 gene never described in the literature. After 3 bleeding episodes, in particular after spontaneous abortion, the diagnosis was confirmed by flow cytometry. This patient was monitored during pregnancy in our centre and then gave birth via vaginal delivery at full term. Special preventive measures were taken. Outcome was good for the mother and the newborn. The treatment of Scott´s syndrome is purely symptomatic and mainly involves platelet transfusions. Since it is a rare thrombopathy with a high bleeding risk and difficult diagnosis, pregnancy and childbirth in patients with this syndrome require rigorous monitoring, as the prognosis is life threatening.

Le syndrome de Scott est une thrombopathie congénitale rare, de transmission autosomique récessive. Notre travail a pour objectif de rapporter le cas d´une parturiente porteuse d´un syndrome de Scott secondaire à une mutation du gène ANO6 jamais décrite dans la littérature. Après 3 épisodes hémorragiques surtout après un avortement spontané, Le diagnostic a été confirmé par une cytométrie en flux. Cette patiente a été suivie tout au long de sa grossesse dans notre formation puis a accouché par voie basse à terme sous mesures préventives particulières avec des suites simples pour la mère et le nouveau-né. Le traitement du syndrome de Scott est purement symptomatique et fait appel principalement aux transfusions de plaquettes. Puisque c´est une thrombopathie rare à haut risque hémorragique, avec un diagnostic difficile, la grossesse et l´accouchement chez les patientes porteuses de ce syndrome nécessitent une surveillance rigoureuse vu que le pronostic vital est engagé.

Keywords: Scott; Thrombopathies; case report; hemorrhage; pregnancy.

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Conflict of interest statement

Les auteurs ne déclarent aucun conflit d´intérêt.

Figures

Figure 1
Figure 1
activation de la phospholipide scramblase
Figure 2
Figure 2
vue schématique de la distribution transversale des phospholipides dans la membrane plasmique des plaquettes au repos et stimulées
Figure 3
Figure 3
chromatogrammes de séquence illustrant la mutation dans la protéine anoctamine 6
Figure 4
Figure 4
cytométrie en flux confirmant l'absence d'expression de plaquettes Scott sur la surface plaquettaire activée
See this image and copyright information in PMC

References

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Supplementary concepts