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Resumen La hemofilia A adquirida es una entidad poco reportada y potencialmente fatal, que se asocia con la aparición de autoanticuerpos contra el factor VIII de la coagulación. Si bien puede estar subestimada, se calcula una incidencia aproximada de 1 a 1.5 casos por millón de habitantes con una mortalidad reportada entre el 9 y el 33%. Se manifiesta con equimosis extensas espontáneas y sangrado en mucosas, tracto gastrointestinal o en el periodo postparto. Se debe sospechar en adultos a partir de la cuarta década de la vida con sangrados espontáneos y un tiempo parcial de tromboplastina prolongado en ausencia de anticoagulante lúpico. Se reporta el caso de un adulto mayor con cardiopatía isquémica, en quien, en el contexto de un evento coronario agudo, se diagnosticó hemofilia A adquirida ante la presencia de sangrado subcutáneo extenso en cuello, con compresión de faringe y laringe que amenazó su vida representando un verdadero reto terapéutico.
Abstract Acquired hemophilia A is an underreported and potentially fatal entity that is associated with the formation of autoantibodies against coagulation factor VIII. Although it may be underestimated, the estimated incidence is between 1-1.5 cases per million people with a reported mortality between 9 and 33%2. It presents with extensive spontaneous ecchymosis, mucosal, gastrointestinal, or postpartum bleeding. It should be suspected in adults from the fourth decade of life with spontaneous bleeding and prolonged TPT in the absence of lupus anticoagulant. We report the case of an older adult with ischemic heart disease in the context of an acute coronary syndrome, who was diagnosed with acquired hemophilia A and presented with significant cervical subcutaneous bleeding with pharyngeal and laryngeal compression that threatened his life, constituting a real therapeutic challenge.
RESUMEN
INTRODUCTION: The etiology of preeclampsia is not fully established. A few studies have shown a relationship between natural coagulation inhibitors and preeclampsia. OBJECTIVES: The purpose of this study was to investigate the status of natural coagulation inhibitors and active protein C resistance (APC-R) in preeclampsia. PATIENTS AND METHODS: We studied 70 women with preeclampsia recruited consecutively and 70 healthy pregnant and 70 nonpregnant women as controls. Plasma protein C (PC), free protein S (fPS), antithrombin III (ATIII) and APC-R were evaluated. RESULTS: ATIII values were found to be significantly lower in preeclamptic patients than in the control groups (p< 0.001). Nevertheless, there was no significant difference between the healthy pregnant and nonpregnant women groups (p=0.141). The fPS values of the preeclamptic and healthy pregnant groups were lower than that of the nonpregnant group (p< 0.001), and the fPS value of the preeclamptic pregnant women was lower than that of healthy pregnant women (p<0.001). The PC value of the preeclamptic pregnant women was lower than that of the control groups (p< 0.001). The PC value of the healthy pregnant women was lower than that of the nonpregnant women (p< 0.001). The mean APC activity values were lower in the preeclamptic patients than that of the control groups (p< 0.001, p< 0.001). The APC-R positivity rates of the preeclamptic groups were higher than that of the control groups (p<0.001). CONCLUSIONS: This study demonstrated that ATIII, fPS, PC values and APC resistance were lower and APC-R positivity was higher in preeclamptic women than in normal pregnant and nonpregnant women.