[ Déficit acquis en facteur V: A propos d’un cas ]
Volume 39, Issue 3, May 2023, Pages 1155–1160
Rachid Elfaiz1, Hanae Bencharef2, Kamana Bella3, Amane Ghita4, Banani Soukaina5, and Bouchra Oukkache6
1 Laboratoire de biochimie, CHU Ibn Rochd de Casablanca, Morocco
2 Hematology Laboratory, Ibn Rochd University Hospital of Casablanca, Morocco
3 Laboratoire d’Hématologie, Centre hospitalier universitaire Ibn Rochd de Casablanca, Morocco
4 Laboratoire d’Hématologie, Centre hospitalier universitaire Ibn Rochd de Casablanca, Morocco
5 Université Hassan II, Faculté de Médecine et de Pharmacie de Casablanca, Morocco
6 Hematology Laboratory, Ibn Rochd University Hospital of Casablanca, Morocco
Original language: French
Copyright © 2023 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.
Factor V deficiency by acquisition of an inhibitor is a rare pathology whose cause is often idiopathic. The clinical phenotype can range from asymptomatic laboratory abnormalities to life-threatening bleeds. We report a case of acquired factor V deficiency diagnosed in our laboratory, which illustrates the diagnostic procedure and the etiological circumstances. Acquired factor V inhibitors develop in extremely rare cases via the development of alloantibodies or autoantibodies against factor V. Several diseases or conditions are associated with factor V inhibitors. In this observation, the predisposing factor was the autoimmune context; our patient has ulcerative colitis. The biological diagnosis of factor V inhibitors (aFVi) remains a challenge, which every biologist must think about when faced with an isolated FV deficiency not explained by hepatic involvement. It is a rare pathology but its occurrence is worrying for both the biologist and the clinician because of the absence of a validated therapeutic strategy.
Author Keywords: Factor V inhibitor, Hemorrhagic syndrome, Autoantibodies, Immunosuppressive therapy, hemostasis.
Volume 39, Issue 3, May 2023, Pages 1155–1160
Rachid Elfaiz1, Hanae Bencharef2, Kamana Bella3, Amane Ghita4, Banani Soukaina5, and Bouchra Oukkache6
1 Laboratoire de biochimie, CHU Ibn Rochd de Casablanca, Morocco
2 Hematology Laboratory, Ibn Rochd University Hospital of Casablanca, Morocco
3 Laboratoire d’Hématologie, Centre hospitalier universitaire Ibn Rochd de Casablanca, Morocco
4 Laboratoire d’Hématologie, Centre hospitalier universitaire Ibn Rochd de Casablanca, Morocco
5 Université Hassan II, Faculté de Médecine et de Pharmacie de Casablanca, Morocco
6 Hematology Laboratory, Ibn Rochd University Hospital of Casablanca, Morocco
Original language: French
Copyright © 2023 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
Factor V deficiency by acquisition of an inhibitor is a rare pathology whose cause is often idiopathic. The clinical phenotype can range from asymptomatic laboratory abnormalities to life-threatening bleeds. We report a case of acquired factor V deficiency diagnosed in our laboratory, which illustrates the diagnostic procedure and the etiological circumstances. Acquired factor V inhibitors develop in extremely rare cases via the development of alloantibodies or autoantibodies against factor V. Several diseases or conditions are associated with factor V inhibitors. In this observation, the predisposing factor was the autoimmune context; our patient has ulcerative colitis. The biological diagnosis of factor V inhibitors (aFVi) remains a challenge, which every biologist must think about when faced with an isolated FV deficiency not explained by hepatic involvement. It is a rare pathology but its occurrence is worrying for both the biologist and the clinician because of the absence of a validated therapeutic strategy.
Author Keywords: Factor V inhibitor, Hemorrhagic syndrome, Autoantibodies, Immunosuppressive therapy, hemostasis.
Abstract: (french)
Le déficit en facteur V par acquisition d’un inhibiteur est une pathologie rare dont la cause reste souvent idiopathique. La présentation clinique des inhibiteurs du facteur V peut aller des formes asymptomatiques à des diathèses hémorragiques graves mettant en jeu le pronostic vital. Nous rapportons un cas de déficit acquis en facteur V diagnostiqué au sein de notre laboratoire, qui illustre la conduite diagnostique ainsi que les circonstances étiologiques. Les inhibiteurs acquis du facteur V se développent dans des cas extrêmement rares via le développement d’allo-anticorps ou d’auto-anticorps contre le facteur V. Leur cause reste souvent imprécise bien que de multiples associations soient rapportées. Dans cette observation, le facteur prédisposant était le contexte auto-immun, notre patient présente une rectocolite hémorragique. Le diagnostic biologique des inhibiteurs du facteur V (aFVi) restent un défi, auquel tout biologiste doit penser devant un déficit isolé en FV non expliqué par l’atteinte hépatique. C’est une pathologie rare mais sa survenue est préoccupante aussi bien pour le biologiste que pour le clinicien vu l’absence de stratégie thérapeutique validée.
Author Keywords: Inhibiteur facteur V, Syndrome hémorragique, Auto-anticorps, Thérapie immunosuppressive, Hémostase.
How to Cite this Article
Rachid Elfaiz, Hanae Bencharef, Kamana Bella, Amane Ghita, Banani Soukaina, and Bouchra Oukkache, “Acquired factor V deficiency: Case report,” International Journal of Innovation and Applied Studies, vol. 39, no. 3, pp. 1155–1160, May 2023.
