CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology.

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Presentation transcript:

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 -7 Dr. BHAVANA MEHTA Consultant pathologist Supratech micropath laboratory and research institute, Ahmedabad SUPRATECH MICROPATH LABORATORY & RESEARCH INSTITUTE

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 MM- Better 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