UNCOMMON SECONDARY POLYCYTHEMIA IN MINIMAL CHANGE DISEASE: CASE REPORT

 

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https://storage.unitedwebnetwork.com/files/1099/1345425ad29e5b69b433b976b193e95e.pdf
UNCOMMON SECONDARY POLYCYTHEMIA IN MINIMAL CHANGE DISEASE: CASE REPORT

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Dr. John Abraham
Tharayil
Dr. John Abraham Tharayil johnabrahamtharayil@gmail.com Ernakulam Medical Centre Nephrology & Renal Transplant Kochi India *
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Polycythaemia associated with Nephrotic Syndrome (NS) is rare but repeatedly reported in case reports and small series. Typical histologies reported include minimal change disease (MCD), focal segmental glomerulosclerosis and membranous nephropathy. Mechanisms are heterogeneous and include (1) true secondary Erythropoetin (EPO)-driven erythrocytosis from intrarenal hypoxia (eg. renal ischemia, renal vein thrombosis), (2) unmasking or coexistence of a primary/myeloproliferative cause (polycythaemia vera, PV), and (3) rarer suggestions such as ectopic EPO or EPO-like factor production or altered EPO regulation/sensitivity. Different patients have shown different patterns (some with elevated serum/urine EPO; others with low EPO and JAK2 mutations consistent with PV). Polycythaemia is defined as increase in red cell mass, reflected by haemoglobin (Hb) concentration more than16.5 g/dL in men and 16 g/dL in women or haematocrit more than 49% in men and 48% in women (WHO 2017). Massive protienuria leads to renal interstitial oedema which compress peritubular capillaries. Hypercoagulable state and local hypoxia increases EPO production and increased red cell mass causing secondary polycythaemia in NS.

This is a case report from Nephrology department of Ernakulam Medical Centre, Kochi, Kerala, India. A 22-year-old male, non-smoker, presented with bilateral lower limb edema for the past 10 days and facial puffiness for the past 2 days. He had a history of fever two months prior, without any associated symptoms. There were no known comorbidities or previous significant medical history. Systemic examination was unremarkable. He was subsequently evaluated with laboratory investigations, abdominal imaging, and a percutaneous renal biopsy.

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Investigations confirmed diagnosis of Nephrotic syndrome with secondary polycythemia. Steroids was initiated and patient had resolution of polycythemia in parallel with remission of NS.

This case report highlights polycythaemia as an unusual association of NS. NS can be associated with secondary erythrocytosis; most evidence comes from case reports/series.  Mechanisms are multiple, most commonly EPO-driven secondary erythrocytosis from intrarenal hypoxia (eg. thrombosis / RVT / micro-ischemia) or, less commonly, coexisting PV. Measurement of serum EPO and JAK2 testing are pivotal first steps. Treat NS and investigate for thrombosis/tumour/PV. Erythrocytosis often improves if the nephrotic syndrome is successfully treated. 

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