Paraneoplastic immunoglobulin A nephropathy and associated focal segmental glomerulosclerosis in asymptomatic low volume B-cell lymphoma - a case report.

ABSTRACT

BACKGROUND: Paraneoplastic glomerulonephritis is rare in haematological malignancies and tends to manifest as minimal change disease, membranous glomerulonephritis or membranoproliferative glomerulonephritis. We present the first report of immunoglobulin A nephropathy and associated focal segmental glomerulosclerosis in a patient with asymptomatic low grade B-cell lymphoma. CASE PRESENTATION A 53 year old gentleman presented with nephrotic range proteinuria (urine protein creatinine ratio of 662 mg/mmol) on a background of type 2 diabetes mellitus (glycosylated haemoglobin < 6%), hypertension, obesity (body mass index 47.6 kg/m2) and degenerative spine disease. Bone marrow biopsy diagnosed a low grade B-cell lymphoma and renal biopsy was consistent with immunoglobulin A nephropathy. Lymphoma treatment with six cycles of cyclophosphamide/ rituximab/ prednisolone led to normalisation of urinary protein excretion (urine protein creatinine ratio 14 mg/mmol at 26 months post-chemotherapy). CONCLUSION: Paraneoplastic immunoglobulin A nephropathy can occur with a broad range of haematological malignancies regardless of stage. This case illustrates the importance of meticulous haematological system work-up for patients presenting with immunoglobulin A nephropathy. Recognition of paraneoplastic immunoglobulin A nephropathy and early diagnosis of associated malignancy can be life-saving.