Focal Segmental Glomerulosclerosis in a Patient with Polycythemia Rubra Vera

R.K. Sharma, MD, Department of Nephrology, Sanjay Gandhi Post-Graduate Institute of Medical Sciences, Raebareli Road, Lucknow 226014 (India) Dear Sir, We report a case of focal segmental glomerulosclerosis (FSGS) with polycythemia rubra vera. The relationship between polycythemia and a renal lesion is discussed. To the best of our knowledge, polycythemia vera in association with FSGS has not been reported so far. A 40-year-old female presented with throbbing headache, giddiness, visual blurring, chest pain and off and on facial flushing of 2 years duration. While undergoing investigations for surgery for a thyroid nodule, the patient was found to have a high hematocrit (Hb 18 g%) count. At that time urine examination did not reveal any proteinuria. One year later she was found to be hypertensive and urine examination showed significant proteinuria (2.0 g in 24 h). On examination the patient was plethoric with no facial or pedal edema, blood pressure was 160/100 mm Hg and the spleen was enlarged. Fundus examination revealed a hyperemic disc and tortuous vessels. Hb was 20.0 g%, hematocrit (Hct) 65%, red blood cell (RBC) count 62×105/cm, total leukocyte count (TLC) 32×103/cm, platelets 4.1×105/cm and the erythrocytic sedimentation rate was 7 mm in the 1 h. Bone marrow was hypercellular and showed erythroid hyperplasia with increased megakaryocytes. 51Cr RBC volume was 38 ml/kg (normal 27-32 ml/kg). Urine examination showed 10-12 RBC/high power field, 6-8 white cells/high power field. Urinary protein excertion was 1.8 g in 24 h. Blood urea nitrogen was 18 mg%, serum creatinine 2.0 mg%, serum uric acid 5.6 mg%, serum albumin 3.2 g% and serum cholesterol 230 mg%. Renal ultrasonography showed normal sized kidneys. Arterial blood gas analysis showed P02 93.6 mm Hg and oxygen saturation of 96.5%. Kidney biopsy was done which showed 15 glomeruli with focally accentuated mesangial proliferation and a mild increase in mesangial matrix, 5 glomeruli showed segmental areas of sclerosis and hya-linosis with synechia formation. Focal areas of tubular atrophy and mild interstitial fibro-sis were seen. Blood vessels displayed intimal fibroelastosis. Biopsy was consistent with FSGS.