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

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Page 1: CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology

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

Page 2: CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology

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

Page 3: CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology

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

Page 4: CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology

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

Page 5: CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology

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

Page 6: CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology

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

Page 7: CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology

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

Page 8: CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology

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

Page 9: CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology

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

Page 10: CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology

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

Page 11: CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology

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

Page 12: CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology

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

Page 13: CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology

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

Page 14: CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology

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

Page 15: CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology

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

Page 16: CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology

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

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

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

Page 18: CME on Renal Pathology, 17th December 08, Pune Indian Society of Renal and Transplantation Pathology in collaboration with Indian Society of Nephrology

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