DOI: 10.12890/2026_007101
Secondary Evans syndrome associated with localized clear cell renal cell carcinoma: a case report and brief literature review
Authors
manerinc1@gmail.com
Keywords
Paraneoplastic syndrome, secondary Evans syndrome, immune thrombocytopenia, renal cell carcinoma, thrombopoietin receptor agonistsAbstract
Introduction: Immune thrombocytopenia (ITP) and autoimmune haemolytic anaemia (AIHA) are uncommon paraneoplastic manifestations of solid tumours, and their association with renal cell carcinoma is exceptional.
Case description: A 66-year-old man with no prior medical history presented with profound thrombocytopenia (5 ×103/µl) and AIHA, confirmed by a strongly positive direct antiglobulin test with IgG and complement. Diagnostic work-up identified a left renal mass corresponding to localized clear cell renal cell carcinoma (pT1b). Bone marrow examination showed preserved megakaryopoiesis without dysplasia or infiltration, and peripheral smear confirmed true thrombocytopenia with macroplatelets. Thrombocytopenia persisted despite corticosteroids, intravenous immunoglobulin, and radical nephrectomy. Sustained platelet recovery was achieved with eltrombopag, a thrombopoietin receptor agonist (TPO-RA).
Conclusion: Secondary Evans syndrome associated with localized renal cell carcinoma is an exceptionally rare disorder. Persistence of cytopenias after surgical tumour control does not rule out a paraneoplastic mechanism, and TPO-RAs may represent an effective option in refractory cases, although evidence in secondary ITP remains limited.
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