Medina-García Gabriela, Ordoñez-González Irvin, Reyes-Navarro Geraldine Vanessa, López-Zamora Berenice, Saavedra Miguel Ángel, Cruz-Domínguez María Pilar, Vera-Lastra Olga
Instituto Mexicano del Seguro Social, Centro Médico Nacional La Raza, Hospital de Especialidades "Dr. Antonio Fraga Mouret", Unidad de Investigación en Medicina Traslacional. Ciudad de México, México.
Universidad Veracruzana, Campus Veracruz, Facultad de Medicina. Veracruz, Veracruz, México.
Rev Med Inst Mex Seguro Soc. 2021 Aug 13;59(3):248-252.
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.
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.
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.
抗磷脂综合征（APS）是一种全身性自身免疫性疾病，其特征为在存在抗磷脂抗体（aPL）的情况下发生动脉或静脉血栓形成和/或产科事件。该病通常在15至50岁的患者中诊断出来，每年每10万人中有5例新发病例。本文报道了一例APS，发生在一名临床表现不寻常的老年患者身上。
一名无自身免疫性疾病病史的女性患者，70岁时出现溶血性贫血，直接抗人球蛋白试验阳性，分类为抗人球蛋白阳性的自身免疫性溶血性贫血（AHAI），以及严重血小板减少症。排除了其他免疫性、感染性、淋巴增殖性疾病和实体瘤。诊断为费希尔 - 埃文斯综合征（FES），治疗反应良好。三个月后，患者左下肢出现深静脉血栓形成，抗磷脂抗体（aPL）阳性，抗心磷脂抗体阳性，确诊为原发性APS，且FES为其初始表现。从那时起，患者一直在接受醋硝香豆素和泼尼松治疗，未再出现血栓复发，仍存在中度血小板减少症，在15年的随访中未出现其他临床表现。
这种疾病在患有合并症的老年人中的不寻常表现不应排除原发性自身免疫性疾病发生的可能性，因此在这个年龄组中应考虑进行诊断。
