Abstract: TH-PO211

Polycythemia Vera: An Unusual Cause of Proteinuria

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Giordano, Christin M., Vanderbilt University Medical Center, Nashville, Tennessee, United States
  • Manolopoulou, Marika, Vanderbilt University Medical Center, Nashville, Tennessee, United States
  • Gould, Ed, Vanderbilt University Medical Center, Nashville, Tennessee, United States
  • Lusco, Mark, Vanderbilt University School of Medicine, Nashville, Tennessee, United States
Background

Introduction: Polycythemia vera (PV) is a myeloproliferative disease which is typpified by increased red cell mass; rarely it has been associated with renal manifestations, including nephrotic syndrome. Here, we present a case of PV associated with glomerular basement membrane thickening and consequent proteinuria.

Methods

Case Description: Patient is a 46 year-old male with a history of mild hypertension on lisinopril monotherapy who presented to nephrology clinic after urinalysis performed as part of a life insurance examination revealed proteinuria. He was otherwise asymptomatic. He was thin and muscular with mild rubot and blood pressure 148/81. Initial laboratory examination was notable for a hematocrit of 56%, creatinine of 1.0 mg/dL, and a urine protein-to-creatinine ratio (PCR) of 3.2 g/g. Renal ultrasound demonstrated normal appearing kidneys but raised concern for hepatatic pathologiy. Magnetic resonance imaging showed mild diffuse hepatic steatosis and splenic vein thrombosis. Laboratory examination was negative except for a mildly postive antinuclear antibody ratio of 1:80. Renal biopsy was pursued. This revealed diffusely thickened glomerular basement membranes concerning for dysmetabolic injury. Given his splenic vein thrombosis and increased hematocrit, he was referred to hematology for PV. He underwent JAK2 mutation testing which was positive. He was initiated on maximal renin-angiotensin-aldosterone blockade and his PCR decreased to 1.9 g/g. As his hematocrit decreased with phlebotomy, his PCR has continued to decrease, most recently to 1.5 g/g.

Conclusion

Discussion: Previous reports have shown an association between myeloproliferative disorders and various glomerulopathies. In the largest published series of these patients, though, only 1 of the 11 reported patients had PV as an explanation for nephrotic syndrome. While the natural history of this condition is uncertain, in a patient being evaluated for new proteinuria with the right clinical findings, PV should be included in the differential diagnosis. Further study of these patients will help us further understand the pathophysiology of the disease and whether treatment of the PV improves renal outcomes.