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      Déficit adquirido de Factor XIII como causa de hemorragia masiva con compromiso de vía aérea superior en cirugía oncológica de cavidad oral Translated title: Acquired Factor XIII deficiency as a cause of massive hemorrhage with upper airway compromise in oncological surgery of the oral cavity

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          Abstract

          RESUMEN El diagnóstico etiológico de una hemorragia postoperatoria persistente en pacientes adultos sin antecedentes médicos de interés supone un reto a día de hoy. Dentro de las distintas causas encontramos el déficit adquirido del Factor XIII (F. XIII) de la coagulación. El déficit de este factor suele pasar desapercibido al no ser valorado en el estudio preoperatorio rutinario. Presentamos el caso de un varón de 70 años con déficit de F. XIII adquirido, no diagnosticado previamente, que tras ser intervenido por un carcinoma epidermoide de suelo de boca sufrió un sangrado de cavidad oral y cervical bilateral con compromiso de la vía aérea.

          Translated abstract

          ABSTRACT The etiological diagnosis of persistent postoperative bleeding in adult patients with no relevant medical history is currently a challenge. Among the different causes, we find the acquired deficiency of Factor XIII (F. XIII) of coagulation. The deficit of this factor usually goes unnoticed as it is not tested in the routine preoperative studies. We present the case of a 70-year-old man with previously undiagnosed acquired F. XIII deficiency who, after undergoing surgery for squamous cell carcinoma of the floor of the mouth, suffered oral and bilateral cervical bleeding causing airway compromise.

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          Most cited references9

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          Factor XIII deficiency.

          Inherited factor XIII (FXIII) deficiency is a rare bleeding disorder that can present with umbilical bleeding during the neonatal period, delayed soft tissue bruising, mucosal bleeding and life-threatening intracranial haemorrhage. FXIII deficiency has also been associated with poor wound healing and recurrent miscarriages. FXIII plays an integral role in haemostasis by catalysing the cross-linking of fibrin, platelet membrane and matrix proteins throughout thrombus formation, thus stabilizing the blood clot. The molecular basis of FXIII deficiency is characterized by a high degree of heterogeneity, which contributes to the different clinical manifestations of the disease. There have been more than 60 FXIII mutations identified in the current literature. In addition, single nucleotide polymorphisms have been described, some of which have been shown to affect FXIII activity, contributing further to the heterogeneity in patient presentation and severity of clinical symptoms. Although there is a lifelong risk of bleeding, the prognosis is excellent when current prophylactic treatment is available using cryoprecipitate or plasma-derived FXIII concentrate.
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            Acquired factor XIII deficiency: a therapeutic challenge.

            Less than 60 cases of acquired factor (F)XIII deficiencies have been reported, most having distinct clinical features. To illustrate the therapeutic challenges of acquired FXIII inhibitors, we report a case of a 65-year-old patient with no previous bleeding history who suddenly developed massive haemorrhages associated to a strong and isolated FXIII inhibitor. No underlying disorder has been detected till now after three years of follow-up. Despite aggressive treatment with prednisone, rituximab, cyclophosphamide, immunoglobulin, immunoadsorption and immune tolerance his inhibitor is still present, although at low titre and with a clinical benefit since the patient has no more bleed since more than one year. Moreover the patient had a venous thromboembolic complication. After a review of the management of acquired FXIII deficiency patients and based on the management of acquired haemophilia we discuss a possible strategy for such difficult cases.
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              Prophylactic and perioperative replacement therapy for acquired factor XIII deficiency.

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                Author and article information

                Journal
                maxi
                Revista Española de Cirugía Oral y Maxilofacial
                Rev Esp Cirug Oral y Maxilofac
                Sociedad Española de Cirugía Oral y Maxilofacial y de Cabeza y Cuello (Madrid, Madrid, Spain )
                1130-0558
                2173-9161
                March 2023
                : 45
                : 1
                : 50-53
                Affiliations
                [1] Santander orgnameHospital Universitario Marqués de Valdecilla orgdiv1Servicio de Cirugía Oral y Maxilofacial España
                Article
                S1130-05582023000100009 S1130-0558(23)04500100009
                10.20986/recom.2023.1428/2023
                95413a5c-07ef-4e34-a8a6-48de9d561d6e

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

                History
                : 28 April 2023
                : 16 February 2023
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 9, Pages: 4
                Product

                SciELO Spain

                Categories
                Casos Clínicos

                compromiso aéreo,Factor XIII,Sangrado postoperatorio,airway compromise,Postoperative bleeding

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