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Secondary Evans Syndrome Associated with Localized Clear Cell Renal Cell Carcinoma: A Case Report and Brief
Eloy Manerinc Peña Monge1,2, Nahilia Carrasco-Randrianarison3, Katherin Ponte-Fernandez1
1Department of Internal Medicine, Hospital Nacional Edgardo Rebagliati Martins, Lima, Peru.
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.
Learning Points:
Secondary Evans syndrome (most commonly concurrent immune thrombocytopenia and autoimmune haemolytic anaemia) is an exceptionally rare paraneoplastic manifestation of localized clear cell renal cell carcinoma and requires rigorous diagnostic exclusion.Persistence of immune cytopenias for several weeks after curative nephrectomy does not exclude a paraneoplastic mechanism, as autoimmune activity may continue beyond tumour control.Thrombopoietin receptor agonists may be effective in immune thrombocytopenia secondary to solid tumours, a scenario with substantially less evidence than primary immune thrombocytopenia.
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