Dr Chawla takes us to a brief overview of plasma cells dyscrasias and the kidney

1. Arun Chawla, MD

3. Copyright ©2010 American Society of Hematology. Copyright restrictions may apply. Ghobrial, I. M. et al. ASH-SAP 2010;2010:581-604 Table 20-1 Diagnostic criteria for monoclonal gammopathies

14. Copyright ©2006 American Society of Nephrology

15. Protein cast obstructs tubule with syncitial giant cell reaction around it

17. Myeloma cast nephropathy demonstrating light-chain restriction in the casts. Copyright ©2006 American Society of Nephrology Korbet, S. M. et al. J Am Soc Nephrol 2006;17:2533-2545 The cast stained with Ig light chain κ (A) shows bright (3+) staining. The same cast stained in a serial section for light chain λ (B) is negative.

21. identify FLCs at a minimum concentration of 100-150mg/l. Insufficient to identify non secretory or oligo-secretory myeloma

22. Technique uses purified polyclonal antibodies against free k and λ light chains which are precoated to latex particles and then analyzed by nephlometric assay for quantification. Permits detection of light chain to a level of 2-4mg/l.

25. Median survival 62 mo Median survival 44 mo Median survival 29 mo

28. EUROPEAN JOURNAL OF CANCER 4 2 ( 2 0 0 6 )

29. Leung et al. KI (2008)

32. Pts got PD in control and HD in PPx group Eleven patients were randomized to receive PLEX. They noted no differences in the overall renal recovery rate however, in a subgroup analysis of the dialysis patients, renal recovery occurred only in those who received PLEX. Small study. compromised by the use of a composite outcome which disadvantaged PLEX because patient who died with improved renal function and those with eGFR <30 were considered failures. the renal pathology was not verified due to the low biopsy rate. Finally, no method was employed to assess the adequacy of treatment.

42. Mesangial & Cap loops amyloid deposits. Arteriolar wall with amyloid.

43. www.pathology.vcu.edu

44. www.pathology.vcu.edu IMMUNOHISTOCHEMICAL STAININGS To differentiate amyloid AL and AA, specific antibodies can also be used.

45. Copyright ©2006 American Society of Nephrology randomly arranged, nonbranching fibrils with an average diameter of 90 to 110 Å Renal amyloidosis, ultrastructural appearance

46. www.pathology.vcu.edu Amyloid p glycoprotein component present in basement membrane binds the amyloid fibrils

56. Copyright ©2006 American Society of Nephrology Korbet, S. M. et al. J Am Soc Nephrol 2006;17:2533-2545 Monoclonal Ig deposition disease (MIDD) with diffuse and nodular glomerulosclerosis

57. Copyright ©2006 American Society of Nephrology Korbet, S. M. et al. J Am Soc Nephrol 2006;17:2533-2545 MIDD showing light chain restriction by immunofluorescence microscopy

58. inconstant electron-dense, subendothelial, granular, punctuate deposits that may diffusely infiltrate the basal lamina.