ISRTPCON 2013 Lt Col Rohit Tewari Dept of Pathology Armed Forces Medical College Pune.

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Presentation transcript:

ISRTPCON 2013 Lt Col Rohit Tewari Dept of Pathology Armed Forces Medical College Pune

Age- 55 yrs Sex- Male Known hypertensive and diabetic (5 yrs) Presented with rapid deterioration of renal function S Cr 1.6 mg% to 7.5 mg% over 4 months. Urine examination- ◦ Alb 2+ ◦ 8-10 pus cells ◦ RBC

S

IgG

C3 C1Q

KappaLambda

Proliferative Glomerulonephritis, Suggestion of Lupus nephritis All serological tests done subsequently for SLE- Neg Renal function progressively worsened over the next one and a half year.

Taken up for Live unrelated renal allograft transplant, standard immunosuppression. Immediate post transplant period – uneventful. Baseline S Cr Brain abscess after 2 months. Mycophenolate stopped.

S Cr 2.3 gm%. Acute graft rejection suspected. Biopsy

S

IgG

C3C1Q

KappaLambda

IgG1IgG2 IgG3IgG4

Proliferative glomerulonephritis with monoclonal immunoglobulin deposits. (PGNMID) Work up for myeloma- initially neg, 2 mths later- M band Recurrent or denovo?

kappaLambda

IgG1 IgG2 iIgG3IgG4

FINAL DIAGNOSIS ◦ Proliferative glomerulonephritis with monoclonal immunoglobulin deposits. ◦ Recurrence in renal allograft. Follow up Autologous Stem cell transplant Doing well reduction in proteinuria

. Kidney International, Vol. 65 (2004), pp. 85–96 Proliferative glomerulonephritis with monoclonal IgG deposits: A distinct entity mimicking immune-complex glomerulonephritis SAMIH H. NASR, GLEN S. MARKOWITZ, M. BARRY STOKES, SURYA V. SESHAN, ELSA VALDERRAMA, GERALD B. APPEL, PIERRE AUCOUTURIER, and VIVETTE D. D’AGATI Department of Pathology and Department of Medicine, Columbia University, College of Physicians and Surgeons, New York, New York; Department of Pathology, Weill Medical College of Cornell University, New York, New York; Department of Pathology, Ten cases described.

Proteinuria in 100% Renal insufficiency in 80% Microhematuria in 60% Monoclonal serum/urinary protein identified in 50% None had evidence of a myeloma/ B cell lymphoproliferative disorder No data on outcome/followup

NDT Plus (2010) 3: 357–359 doi: /ndtplus/sfq076 Advance Access publication 2 May 2010 Case Report Steroid-responsive nephrotic syndrome in a patient with proliferative glomerulonephritis with monoclonal IgG deposits with pure mesangial proliferative features Atsushi

One patient who had denovo disease in the allograft One patient had recurrent disease 1 yr after transplant 1503 Proliferative Glomerulonephritis with Monoclonal IgG Deposits Recurs or May Develop De Novo in Renal Allografts A Albawardi, A Satoskar, S Brodsky, GM Nadasdy, T Nadasdy. The Ohio State University,

Why this case is presented? Rarity of the condition Early recurrence in the renal allograft Importance of routinely performing kappa and lambda in renal biopsy. Possibility of initial negativity of myeloma workup. Recognizing and interpreting linear accentuation in diabetes.