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SUPRATECH MICROPATH LABORATORY & RESEARCH INSTITUTE Case -7 Dr. BHAVANA MEHTA Consultant pathologist Supratech micropath laboratory and research institute, Ahmedabad CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology (ISN) -ISNCON 2008 Clinical details • • • • 38 yrs, male Renal transplant-5 yrs back On cyclosporin ,Azoran, steroids S cr- gradually increased upto 3.0, urine protein++ • Renal biopsy done CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology (ISN) -ISNCON 2008 CASE 1 • CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology (ISN) -ISNCON 2008 • CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology (ISN) -ISNCON 2008 • CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology (ISN) -ISNCON 2008 • CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology (ISN) -ISNCON 2008 CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology (ISN) -ISNCON 2008 Provisional Diagnosis Post transplant myeloma cast nephropathy CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology (ISN) -ISNCON 2008 Why cast nephropathy in post Tx Allograft nephropathy ARF Drug related cast CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology (ISN) -ISNCON 2008 Pathogenesis of myeloma cast nephropathy Common in multiple myeloma Due to over production and precipitation of light chain Resistant to degradation due to structural changes Increased urinary calcium due to hypercalcemia Acute renal failure due to other disease (volume depletion Drug related –loop diuretics by increasing luminal NaCl Radiocontrast media interacting with light chains CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology (ISN) -ISNCON 2008 What are myeloma casts? • Precipitated light chains in distal and collecting tubules +other filtered proteins+Tamm-Horsfall mucoprotein which a protein normally secreted by cells of thick ascending loop of Henle and constitute matrix of all urinary casts • Disruption of BM of tubule-leakage-cellular reaction CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology (ISN) -ISNCON 2008 Other disease with myeloma casts • • • • Acinar cell carcinoma, pancreas Waldenstrom macroglobulinemia B cell lymphomas Thyroid carcinoma CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology (ISN) -ISNCON 2008 History retrieved •H/o basic disease-Myeloma kidney diagnosed in 2001 •Urine-Bence John’s protein present •Protein electrophoresis –M band present CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology (ISN) -ISNCON 2008 Final diagnosis Relapse of myeloma cast nephropathy, renal graft. Donor-sister No family history Post transplant period-no problem till 5 yrs CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology (ISN) -ISNCON 2008 Post Tx myeloma cast nephropathy • • • • Usually Tx not considered in myeloma pt. Becasuse of risk of recurrence & relapse If in remission for 1 yr, considered for Tx Usually recurrence noted within 2 yrs CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology (ISN) -ISNCON 2008 Post Tx myeloma cast nephropathy • One case reported in early transplant period associated with thrombotic microangiopathy • Upto 10 cases of MM and few with AL amyloidosis received Tx- more post Tx mortality • With recent treatments available for MMBetter post Transplant period CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology (ISN) -ISNCON 2008 Reasons to present this case Young Patient to have MM No Bone marrow involvement Intraabdominal mass-reported as Multiple myeloma Late graft dysfunction-5 yrs Presently on Thalidomide and doing well patient CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology (ISN) -ISNCON 2008 CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology (ISN) -ISNCON 2008