Abstract: FR-PO1281
Renovascular Hypertension from Bilateral Renal Artery Occlusion/Stenosis in Triple-Negative Essential Thrombocythemia: A Case Report
Session Information
- Onconephrology: Diagnostic Dilemmas, Therapy-Related Toxicities, and Clinical Cases
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Onconephrology
- 1600 Onconephrology
Authors
- Brito, Germana Alves, A C Camargo Cancer Center, São Paulo, SP, Brazil
- Pereira, Benedito J., A C Camargo Cancer Center, São Paulo, SP, Brazil
- Fernandes, Lizieux Matos, A C Camargo Cancer Center, São Paulo, SP, Brazil
- Baptista, Aline Lourenco, A C Camargo Cancer Center, São Paulo, SP, Brazil
- Krutman, Mariana, A C Camargo Cancer Center, São Paulo, SP, Brazil
- Imanishe, Marina Harume, A C Camargo Cancer Center, São Paulo, SP, Brazil
- Andrade, Luis Andre, A C Camargo Cancer Center, São Paulo, SP, Brazil
- Kataoka, Paulo Vitor, A C Camargo Cancer Center, São Paulo, SP, Brazil
Introduction
Essential thrombocythemia (ET) is a myeloproliferative neoplasm associated with arterial thrombosis and microvascular dysfunction. Macrovascular renal artery involvement is a rare complication manifesting as severe hypertension and progressive kidney dysfunction
Case Description
A 49-year-old man with persistent thrombocytosis identified during evaluation of migraine with visual aura developed new-onset severe hypertension and chronic kidney disease. Platelet count was 640,000/mm3, eGFR 38 mL/min/1.73 m2, and mild proteinuria (218mg/24h) was present. Duplex ultrasonography revealed left renal atrophy with tardus–parvus waveforms and peak systolic velocity of 315 cm/s in the proximal right renal artery. Magnetic resonance angiography demonstrated complete left renal artery occlusion and high-grade proximal right renal artery stenosis, with multifocal celiac trunk stenoses in the absence of systemic atherosclerosis. Bone marrow biopsy confirmed megakaryocytic hyperplasia; a myeloid gene panel identified an MPL hotspot mutation and TET2 deletion. JAK2 V617F, CALR, and MPL driver mutations were negative, establishing triple-negative ET. Aspirin and hydroxyurea were initiated. Percutaneous angioplasty with right renal artery stenting resulted in improved blood pressure control and recovery of kidney function (eGFR 62 mL/min/1.73 m2). Clinical and imaging findings are summarized in the Figure.
Discussion
Prior reports of renovascular occlusion in ET involve predominantly JAK2 V617F-positive patients. This case extends that association to triple-negative ET, demonstrating that macrovascular renal disease may occur across the full molecular spectrum of ET, including its rarest subtype. In patients with a solitary functioning kidney, early revascularization combined with optimized hematologic therapy may prevent irreversible renal loss. Severe hypertension and kidney dysfunction in ET — regardless of driver mutation status — should prompt systematic evaluation for renovascular disease.