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A case report and literature review of IgA nephropathy presenting as nephrotic syndrome in polycythemia vera

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Abstract

A 66-year-old non-smoker presented with a 2-week history of new-onset pedal oedema and gross haematuria. On evaluation, he was found to be hypertensive and oedematous with a haemoglobin of 19.1 g/dl, platelet count of 546,000/mm3, and creatinine of 2.6 mg/dl. Urine examination revealed abundant RBCs with 3+ albumin on three separate occasions. His 24-h urine protein level was 3830 mg/day, with a serum cholesterol level of 303 mg/dl. Secondary erythrocytosis and thrombocytosis tests were negative. Bone marrow examination revealed hypercellularity, erythroid hyperplasia, tight clusters of large megakaryocytes, and megakaryocytic hyperplasia suggestive of polycythemia vera. PCR analysis revealed a JAK2V617 F (exon 14) mutation. In view of nephrotic syndrome, azotemia, and microscopic haematuria, a renal biopsy was performed, which revealed features of IgA nephropathy with advanced interstitial fibrosis and tubular atrophy. He was started on angiotensin receptor blockers with hydroxy urea as a part of treatment. This case report highlights the association of glomerular disease with polycythaemia vera and the need of prompt renal biopsy for diagnosis and management.

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Acknowledgements

The authors acknowledge the wholehearted encouragement of Dr .S Illango, Associate nephrology in Nephrology for this case report.

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There was no financial grant for this case report.

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Authors and Affiliations

  1. Department of Nephrology, SRM Medical College Hospital and Research Centre, Kattankulathur, Tamil Nadu, 603203, India

    R. Rajasekar, Gerry George Mathew & V. Jayaprakash

  2. Department of General Medicine, SRM Medical College Hospital and Research Centre, Kattankulathur, Tamil Nadu, 603203, India

    R. Nandakumar, Saurav P. Singhvi & K. Mythili

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  1. R. Rajasekar
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  2. R. Nandakumar
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  3. Saurav P. Singhvi
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  4. Gerry George Mathew
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  5. V. Jayaprakash
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  6. K. Mythili
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Contributions

R. Rajasekar , R Nanda Kumar , Saurav P Singhvi , Gerry George Mathew ,V Jayaprakash and K Mythili were responsible for conception, design, visualisation, investigation, drafting of the text, sourcing and editing of clinical images, critical revision of important intellectual content and gave final approval of the manuscript.

Corresponding author

Correspondence to Gerry George Mathew.

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The authors declare that they have no competing interests.

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This case report was drafted after written informed consent of the patient in conformity with CARE clinical case reporting guidelines and Declaration of Helsinki. The authors are accountable for all aspects of the work (including full data access, integrity of the data, and accuracy of the data analysis) to ensure that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

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Rajasekar, R., Nandakumar, R., Singhvi, S.P. et al. A case report and literature review of IgA nephropathy presenting as nephrotic syndrome in polycythemia vera. CEN Case Rep (2024). https://doi.org/10.1007/s13730-024-00879-x

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