Bilateral primary renal diffuse large B-cell lymphoma: a rare presentation of paediatric renal disease mimicking juvenile nephronophthisis

Bruce, G., Chaudhury, S. and Reynolds, B. (2020) Bilateral primary renal diffuse large B-cell lymphoma: a rare presentation of paediatric renal disease mimicking juvenile nephronophthisis. BMJ Case Reports, 13(2), e234810. (doi: 10.1136/bcr-2020-234810) (PMID:32675115)

Abstract

A 12-year-old boy presented with a prolonged history of headache, fatigue and hypertension. Initial investigations were consistent with presumed non-oliguric end-stage renal disease, leading to a provisional diagnosis of juvenile nephronophthisis. Subsequent imaging demonstrated bilaterally enlarged kidneys without cystic change. Mutation analysis was negative for nephronophthisis, causing diagnostic uncertainty which prompted renal biopsy. Histology revealed a primary renal diffuse large B-cell lymphoma which was highly responsive to chemotherapy, including the anti-CD20 monoclonal agent, rituximab. Renal function improved during lymphoma treatment, with residual chronic kidney disease stage 3a once chemotherapy was completed. Atypical diagnostic features should always prompt re-evaluation of a patient. In this case, the delayed malignancy diagnosis did not have an adverse effect on patient survival or morbidity. The outcome for primary renal lymphoma (PRL) has improved markedly following the introduction of rituximab.

• Item Type: Articles
• Status: Published
• Refereed: Yes
• Glasgow Author(s) Enlighten ID: Chaudhury, Dr Shahzya and Bruce, Dr Gordon and Reynolds, Dr Ben
• Authors: Bruce, G., Chaudhury, S., and Reynolds, B.
• College/School: College of Medical Veterinary and Life Sciences > School of Cancer Sciences; College of Medical Veterinary and Life Sciences > School of Medicine, Dentistry & Nursing
• Journal Name: BMJ Case Reports
• Publisher: BMJ Publishing Group
• ISSN: 1757-790X
• ISSN (Online): 1757-790X
• Copyright Holders: Copyright © BMJ Publishing Group Limited 2020
• First Published: First published in BMJ Case Reports 13(2):e234810
• Publisher Policy: Reproduced in accordance with the publisher copyright policy