Nephrotic Syndrome as the Initial Presentation of Polycythemia Vera

Case Report

Austin J Nephrol Hypertens. 2015;2(2): 1035.

Nephrotic Syndrome as the Initial Presentation of Polycythemia Vera

Shehab T1* and Ismail W2

1Department of Nephrology, Cairo Kidney Center, Egypt

2Department of Pathology, Medical School of BaniSuef University, Egypt

*Corresponding author: Shehab T, Department of Nephrology &Cairo Kidney Center, POB 91 Bab el Louk 11513, Cairo, Egypt

Received: December 01, 2014; Accepted: February 09, 2015; Published: February 11, 2015


Polycythemia Vera as an entity of Myleproliferative Neoplasm (MPNs) has infrequent renal involvement which is usually late and rarely recognized. It is characterized clinically by nephrotic range proteinuria and renal insufficiency. The histological pattern of affection is mostly glomerular and is characterized by a combination of mesangial sclerosis, hypercellularity, segmental sclerosis and features of chronic Thrombotic Microangiopathy (TMA). Clinical and histological awareness is needed to establish diagnosis and achieving possible reversibility

Keywords: Polycythemia vera; Myeloproliferative; Glomerulopathy; Nephrotic syndrome

Case Presentation

A 60 year-old female patient presented with progressive facial and lower limb edema of 5 months duration. She has been hypertensive since 6 years and is currently receivingamlodipine10 mg /day. Her medical history was significant for intermittent attacks of dark bluish discoloration of the fingers and toes for the past 3 years. This was associated with parathesia and tingling sensation not related to cold exposure and not associated with any pain. These attacks were also associated with localized pruritus which occasionally became generalized, upon which she was diagnosed as “skin allergy” , for which she was prescribed antihistamines, yet with a poor response. She had occasional epistaxis that was attributed to uncontrolled blood pressure.

She was married with healthy off springs, retired, and physically active with irrelevant family history.

Upon examination she looked apathetic but well oriented to time and place. Her blood pressure was 160/90 mm Hg. Her vital signs were within normal. Abdominal examination revealed a huge spleen crossing the midline. There was moderate below knee pitting lower limb oedema and scratch marks, mainly on her arms. No other abnormalities were detected.

Spot urine analysis showed proteinuria with 5gm protein/ gm creatinine. Serum creatinine was 1.8 mg/dl urea 88mg/dl and uric acid 10.2mg/dl. Her complete blood picture revealed mild thrombocytosis and neutrophilia without remarkable leuckocytosis. Virology and immunology profile were negative (Table 1).