Síndrome antifosfolípido primario con debut tardío en el adulto mayor / Late onset primary antiphospholipid syndrome in the older adult
Palabras clave:
Síndrome Antifosfolípido, Anciano, Enfermedades de Inicio Tardío / Antiphospholipid Syndrome, Aged, Late Onset DisordersResumen
Resumen
Introducción: el síndrome antifosfolípido (SAF) es una enfermedad autoinmune sistémica, caracterizada por trombosis arterial o venosa, o eventos obstétricos en presencia de anticuerpos antifosfolípidos (aPL). Suele diagnosticarse entre los 15 y los 50 años, y hay cinco casos nuevos por cada 100 000 personas al año. Se reporta un caso de SAF que presenta una adulta mayor con manifestación clínica poco usual.
Caso clínico: paciente mujer, sin antecedentes de enfermedades autoinmunes, que a los 70 años presentó anemia hemolítica y Coombs directo positivo, lo cual se catalogó como anemia hemolítica autoinmune (AHAI) Coombs+, y trombocitopenia severa. Se descartaron otros trastornos inmunológicos, infecciosos, linfoproliferativos y tumores sólidos, y se diagnosticó síndrome de Fisher-Evans (SFE) con buena respuesta al tratamiento. Tres meses después, la paciente presentó trombosis venosa profunda en miembro pélvico izquierdo. Se determinaron aPL positivos y aloanticuerpos positivos, y se estableció el diagnóstico de SAF primario y como su manifestación inicial el SFE. Desde entonces la paciente fue tratada con acenocumarina y prednisona sin recurrencias de trombosis, con persistencia de trombocitopenia moderada y sin nuevas manifestaciones clínicas en 15 años de seguimiento.
Conclusión: ante la presentación inusual de esta enfermedad en adultos mayores con comorbilidades no se debe descartar la posibilidad del desarrollo de una enfermedad autoinmune primaria, por lo cual se debe tener en cuenta para su diagnóstico en este grupo etario.
Abstract
Background: Antiphospholipid syndrome (APS) is a systemic autoimmune disease, characterized by arterial or venous thrombosis and/or obstetric events in the presence of antiphospholipid antibodies (aPL). It is usually diagnosed in patients between the ages of 15 and 50 years, and there are 5 new cases per 100,000 people per year. It is reported a case of APS, which it is present in an older adult with an unusual clinical manifestation.
Clinical case: Female patient without history of autoimmune diseases, at age 70 presented hemolytic anemia, Coombs direct positive, classified as autoimmune hemolytic anemia (AHAI) Coombs+, and severe thrombocytopenia. Other immunological, infectious, and lymphoid proliferative disorders and solid tumors were ruled out. Fisher-Evans syndrome (FES) was diagnosed with good response to treatment. Three months later, the patient presented deep venous thrombosis in the left pelvic limb, positive antiphospholipid antibodies (aPL) and positive aloantibodies were determined, establishing the diagnosis of primary APS and FES as its initial manifestation. Since then, the patient has been in treatment with acenocoumarol and prednisone without new recurrences of thrombosis, with persistence of moderate thrombocytopenia, without adding another clinical manifestation in 15 years of follow-up.
Conclusion: The unusual presentation of this disease in older adults with comorbidities should not rule out the possibility of the development of a primary autoimmune disease, so it should be considered for diagnosis in this age group.
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