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

BMC Nephrol. 2018 Sep 10;19(1):224. doi: 10.1186/s12882-018-1034-y.

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.

Keywords: Glomerulonephritis; Immunoglobulin A nephropathy; Lymphoma; Non-Hodgkin; Paraneoplastic; Proteinuric.

Publication types

  • Case Reports

MeSH terms

  • Asymptomatic Diseases*
  • Glomerulonephritis, IGA / complications
  • Glomerulonephritis, IGA / diagnosis*
  • Glomerulonephritis, IGA / urine
  • Glomerulosclerosis, Focal Segmental / complications
  • Glomerulosclerosis, Focal Segmental / diagnosis*
  • Glomerulosclerosis, Focal Segmental / urine
  • Humans
  • Lymphoma, B-Cell / complications
  • Lymphoma, B-Cell / diagnosis*
  • Lymphoma, B-Cell / urine
  • Male
  • Middle Aged