Paraneoplastic ANCA positive crescentic glomerulonephritis as presenting feature of Mantle Cell lymphoma a unique case.

INTRODUCTION

Mantle Cell Lymphoma is a rare Non-Hodgkin, with unprecedented kidney involvement

MATERIAL

case report

OBSERVATIONS

Herein IPGMER-SSKM Kolkata a 57-year-old male presented to us with Low-grade-intermittent fever & B-symptoms of 4months, arthralgia of 3months & oliguria of 10days. Mild pallor, mild hepatosplenomegaly & significant generalized lymphadenopathy. Hemoglobin 8.7% ESR 106 Uric acid 8.5 Potassium 6.3 Phosphate 4.5 Calcium 6.5 Urea 193 Creatinine 15 indicated Tumour Lysis Syndrome. Bilateral 10 cm kidney with Renal Parenchymal disease in USG. 25 RBC/hpf Albumin 3+ in Urine Study. 4.55gm protein per 24-hour-urine. Negative C3, C4, RF, anti-CCP, dsDNA. but 4+ ANCA, high 170 PR3. Crescents in 5 out of 8 glomeruli, focal interstitial lymphoid aggregates & (IgG, C3, C1q, IgM) positive Immune Complexes in Renal Biopsy. (CD20, CD5, BCL2, Cyclin-D1) Positive Mantle cell Lymphoma in Cervical Lymph Node Biopsy with 10% Ki67 index, High 6.18 MIPI score. R-CHOP regimen & Successive Hemodialysis improved condition of the patient. Discussion: paraneoplastic glomerulonephritis & direct lymphocytic infiltration lead to RPGN in our patient

CONCLUSIONS

To our knowledge, this is possibly first case report of a Mantle cell lymphoma presenting with RPGN due to both ANCA positive crescentic glomerulonephritis & lymphoid cell infiltration of Interstitium.