Clinicopathologic Assessment of Monoclonal Immunoglobulin (Ig) Associated Renal Disease in the Kidney Allograft
1Nephrology, Columbia Unversity Medical Center, NEW YORK, NY, 2Nephrology, Hartford Hospital, Hartford, CT, 3Surgery, Christiana Hospital, Newark, DE, 4Pathology, Columbia Unversity Medical Center, NEW YORK, NY
Meeting: 2019 American Transplant Congress
Abstract number: C81
Keywords: Biopsy, Lymphoproliferative disease
Session Information
Date: Monday, June 3, 2019
Session Name: Poster Session C: Kidney Complications: Late Graft Failure
Session Time: 6:00pm-7:00pm
Presentation Time: 6:00pm-7:00pm
Location: Hall C & D
*Purpose: Monoclonal (M)Ig-associated renal disease comprises a group of disorders caused by paraproteins. Little is known about this entity in the renal allograft, as it is rarely encountered and poorly studied.
*Methods: We retrospectively searched our archives for renal allograft biopsies diagnosed with MIg-associated renal disease from 2007-2018.
*Results: The cohort included 26 patients with the following diagnoses: AL amyloidosis (n=5), light chain deposition disease (n=5), light chain proximal tubulopathy (n=2), light chain cast nephropathy (n=1), and proliferative glomerulonephritis with MIg deposits (PGNMID) (n=13). Patients were 54 ± 16 years old and included 31% women and 16% black race. MIg-associated renal disease accounted for 9 (35%) of ESRD etiologies in the native kidney while the others were presumed to have manifested de novo. The disease occurred on average 5.4 ± 5.4 years after transplantation. Thirteen (50%) patients had hematologic disorders: 7 multiple myeloma, 1 non-Hodgkin lymphoma, and 5 plasma cell neoplasm (bone marrow cellularity <10%). In 8 (62%) of these 13 patients, the allograft biopsy diagnosis triggered a hematologic workup and the new detection of hematologic disorder.
Patients with PGNMID (n=13) were compared to the group with all other MIg-related conditions combined (n=13). While graft survival was similar, PGNMID patients had lower frequency of detectable M-spike in the serum or urine [3/13 (23%) vs 12/13 (92%) P=0.001] and overt hematologic disorder [3/13 (23%) vs 10/13 (77%) P=0.02]. Allograft biopsies of all 3 PGNMID patients with hematologic disorders demonstrated organized deposits [2 fibrils; 1 lattice-like vs. 0 other PGNMID] and none had IgG3 staining (vs 40% in other PGNMID). In 6/13 (46%) patients with PGNMID, the clonality of the deposits changed from monoclonal to polyclonal or vice versa between native kidney and allograft (n=3) or on serial allograft biopsies (n=3).
*Conclusions: In conclusion, post-kidney transplant PGNMID often lacks an identifiable hematologic disorder, especially in cases with IgG3, undetectable M-spike, and no deposit substructure. In such cases, careful follow-up by serial serum and urine immunofixation may be sufficient. In contrast, a thorough hematologic workup is needed in other forms of MIg-associated renal disease.
To cite this abstract in AMA style:
Kamal J, Crew RJ, Ye X, Swanson J, Appel G, Markowitz G, D'Agati V, Batal I. Clinicopathologic Assessment of Monoclonal Immunoglobulin (Ig) Associated Renal Disease in the Kidney Allograft [abstract]. Am J Transplant. 2019; 19 (suppl 3). https://atcmeetingabstracts.com/abstract/clinicopathologic-assessment-of-monoclonal-immunoglobulin-ig-associated-renal-disease-in-the-kidney-allograft/. Accessed April 20, 2021.« Back to 2019 American Transplant Congress