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Síndrome antifosfolípido primario con debut tardío en el adulto mayor / Late onset primary antiphospholipid syndrome in the older adult

Gabriela Medina-García, Irvin Ordoñez-González, Geraldine Vanessa Reyes Navarro, Berenice López-Zamora, Miguel Ángel Saavedra, María Pilar Cruz-Domínguez, Olga Vera-Lastra



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.



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.


Palabras clave

Síndrome Antifosfolípido; Anciano; Enfermedades de Inicio Tardío / Antiphospholipid Syndrome; Aged; Late Onset Disorders

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Grimaud F, Yelnik C, Pineton de Chambrun M, Amoura Z, Arnaud L, Costedoat-Chalumeau N, et al. Clinical and immunological features of antiphospholipid syndrome in the elderly: a retrospective national multicentre study. Rheumatology (Oxford). 2019; 58(6):1006-10.


Cervera R. Antiphospholipid syndrome. Thromb Res. 2017;151(1):43-7.


Ghembaza A, Saadoun D. Management of Antiphospholipid Syndrome. Biomedicines. 2020;8(11):508.


Cervera R, Serrano R, Pons-Estel GJ, Ceberio-Hualde L, Shoenfeld Y, De Ramón E, et al. Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: a multicentre prospective study of 1000 patients. Ann Rheum Dis. 2015;74(6):1011-8.


Shaikh H, Mewawalla P. Evans Syndrome. In: StatPearls [Internet]. Florida: Treasure Island; 2020 Jun 23.


Michel M, Chanet V, Dechartres A, Morin AS, Piette JC, Cirasino L, et al. The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases. Blood. 2009;114(15):3167-72.


Seif AE, Manno CS, Sheen C, Grupp SA, Teachey DT. Identifying autoimmune lymphoproliferative syndrome in children with Evans syndrome: a multi-institutional study. Blood. 2010;115(11):2142-5.


Asherson RA, Khamashta MA, Ordi-Ros J, Derksen RH, Machin SJ, Barquinero J, et al. The “primary” antiphospholipid syndrome: major clinical and serological features. Medicine (Baltimore). 1989;68(6):366-74.


Comellas-Kirkerup L, Hernández-Molina G, Cabral AR. Antiphospholipid-associated thrombocytopenia or autoimmune hemolytic anemia in patients with or without definite primary antiphospholipid syndrome according to the Sapporo revised classification criteria: a 6-year follow-up study. Blood. 2010;116(16):3058‑63.


Cervera R, Boffa MC, Khamashta MA, Hughes GR. The Euro-Phospholipid project: epidemiology of the antiphospholipid syndrome in Europe. Lupus. 2009;18(10):889-93.


Jaime-Pérez JC, Aguilar-Calderón PE, Salazar-Cavazos L, Gómez-Almaguer D. Evans syndrome: clinical perspectives, biological insights and treatment modalities. J Blood Med. 2018;10(9):171‑84.


Audia S, Grienay N, Mounier M, Michel M, Bonnotte B. Evan’s syndrome: from diagnosis to treatment. J Clin Med. 2020;9(12):3851.


Stolyar L, Rizi B, Lin S, Hsieh E. Systemic lupus erythematosus presenting as Evans syndrome. Clin Case Rep. 2019; 7:2513‑4.


Zulfiqar AA, Mahdi R, Mourot-Cottet R, Pennaforte JL, Novella JL, Andrès E. Autoimmune hemolytic anemia – a short review of the literature, with a focus on elderly patients. J Hematol Thrombo Dis. 2015;03(06):228.


Barcellini W, Fattizzo B. The changing landscape of autoimmune hemolytic anemia. Front Immunol. 2020;11:946.


Cervera R, Piette JC, Font J, Khamashta MA, Shoenfeld Y, Camps MT, et al. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum. 2002;46:1019‑27.


Cherif Y, Jallouli M, Hriz H, Gouiaa R, Marzouk S, Snoussi M, et al. Late-onset primary antiphospholipid syndrome in the elderly: a report of seven cases. Int J Rheum Dis. 2015;18(1):103‑7.


Sciascia S, Amigo MC, Roccatello D, Khamashta M. Diagnosing antiphospholipid syndrome: 'extra-criteria' manifestations and technical advances. Nat Rev Rheumatol. 2017;13(9):548‑60.


Manukyan D, Rossmann H, Schulz A, Zeller T, Pfeiffer N, Binder H, et al. Distribution of antiphospholipid antibodies in a large population-based German cohort. Clin Chem Lab Med. 2016;54:1663‑70.


Mahaum N, Prete PE. Antiphospholipid antibodies in malignancy: are these pathogenic or epiphenomena? J Clin Rheumatol. 2011;17(8):429-31.


Pham C, Shen YM. Antiphospholipid antibodies and malignancy. Hematol Oncol Clin North Am. 2008;22:121-30.


Sawamura M, Yamaguchi S, Murakami H, Amagai H, Matsushima T, Tamura J, et al. Multiple autoantibody production in a patient with splenic lymphoma. Ann Hematol. 1994;68(5):251‑4.


Thiagarajan P, Shapiro S, De Marco L, Monoclonal immunoglobulin M lambda coagulation inhibitor with phospholipid specificity. Mechanism of a lupus anticoagulant. J Clin Invest.1980;66(3):397‑405.


Zuckerman E, Toubi E, Golan TD, Rosenvald-Zuckerman T, Sabo E, Shmuel Z, et al. Increased thromboembolic incidence in anti-cardiolipin-positive patients with malignancy. Cancer. 1995;72(2):447.


Lip GY, Chin BS, Blann AD. Cancer and the prothrombotic state. Lancet Oncol. 2002;3(1):27‑34.


Prandoni P, Falanga A, Piccioli A. Cancer and venous thromboembolism. Lancet Oncol. 2005;6(6):401‑10.


Gouin-Thibault I, Achkar A, Samama M. The thrombophilic state in cancer patients. Acta Haematol. 2001;106(1-2)33‑42.


Islam MA, Alam F, Sasongko TH, Siew HG. Antiphospholipid antibody-mediated thrombotic mechanisms in antiphospholipid syndrome: towards pathophysiology-based treatment. Curr Pharm Des. 2016;22(28):4451‑69.


Schreiber K, Sciascia S, De Groot PG, Devreese K, Jacobsen S, Ruiz-Irastorza G, et al. Antiphospholipid syndrome. Nat Rev Dis Primers. 2018;4:17103.

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