cme on renal pathology, 17th december 08, pune indian society of renal and transplantation pathology...
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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
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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
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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 nephropathyARFDrug 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-sisterNo family historyPost 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 MMNo Bone marrow involvementIntraabdominal mass-reported as Multiple
myelomaLate graft dysfunction-5 yrsPresently 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