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, 13 (7), 1037-1046

Kidney Involvement of Patients With Waldenström Macroglobulinemia and Other IgM-Producing B Cell Lymphoproliferative Disorders

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Kidney Involvement of Patients With Waldenström Macroglobulinemia and Other IgM-Producing B Cell Lymphoproliferative Disorders

Larissa Higgins et al. Clin J Am Soc Nephrol.

Abstract

Background and objectives: Kidney involvement in Waldenström macroglobulinemia is less well described compared with kidney manifestations in multiple myeloma.

Design, setting, participants, & measurements: Of the 1363 patients seen with Waldenström macroglobulinemia and other IgM-secreting B cell lymphoproliferative disorders seen at the Mayo Clinic between 1996 and 2015, 57 kidney biopsies were retrospectively studied. The biopsy findings were correlated with clinical, kidney, and hematologic characteristics. Criteria for inclusion were evidence of a monoclonal IgM protein and availability of a kidney and a bone marrow biopsy for review. Glomerular and tubulointerstitial pathologies were categorized according to whether they were related to the monoclonal IgM.

Results: Of the 57 patients identified, monoclonal gammopathy-related kidney lesions were identified in 82% (47 of 57 biopsies), whereas nonmonoclonal gammopathy-related kidney lesions were seen in 18% (ten of 57). Monoclonal gammopathy-related kidney lesions included monoclonal Ig-related amyloidosis (n=19; 33%), nonamyloid glomerulopathy (n=20, 35%), and tubulointerstitial nephropathies (n=8; 14%). The most common monoclonal gammopathy-related kidney lesion was monoclonal Ig-related amyloidosis (n=19; 33%) followed by cryoglobulinemic GN (n=13; 28%). Lymphoma infiltration was the most common tubulointerstitial lesion (n=4; 9%). The hematologic diagnosis was Waldenström macroglobulinemia in 74% (n=42), monoclonal gammopathy of renal significance in 16% (n=9), and marginal zone lymphoma (n=2), chronic lymphocytic leukemia (n=2), and low-grade B cell lymphoma (n=2) in 4% each.

Conclusions: Our study confirms a diverse variety of kidney lesions in patients with monoclonal IgM gammopathy.

Keywords: Adult; Amyloidosis; B-Lymphocytes; Biopsy; Bone Marrow; Humans; IgM; Immunoglobulin M; Lymphoma, B-cell; Lymphoproliferative; MGRS; Monoclonal Gammopathy of Undetermined Significance; Myeloma Proteins; Paraproteinemias; Retrospective Studies; Waldenstrom Macroglobulinemia; glomerular disease; glomerulonephritis; multiple myeloma.

Figures

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Figure 1.
Figure 1.
Cryoglobulinemic GN. Cryoglobulinemic GN is the most common nonamyloid glomerulopathy in this series. (A) The glomerulus shows a membranoproliferative GN pattern of injury. There is global mesangial and endocapillary hypercellularity, numerous intracapillary infiltrating monocytes and some neutrophils, and duplication of the glomerular basement membranes associated with cellular interposition (periodic acid–Schiff stain). Magnification, ×200. (B) An electron microscopy figure from the same case showing small mesangial electron deposits composed of ill-defined short curved or straight microtubules. Magnification, ×20,000.
Figure 2.
Figure 2.
Proliferative GN with monoclonal Ig deposits, IgM variant. This figure describe an IgM variant of the proliferative GN with monoclonal Ig deposits which was initially described only with monoclonal IgG deposits. (A) Light microscopy exhibits a mesangial proliferative GN pattern of injury (periodic acid–Schiff stain). (B) On electron microscopy, granular mesangial and subendothelial electron dense deposits (without substructure) are seen. On immunofluorescence, there was bright granular glomerular mesangial positivity for (C) IgM and (D) κ-light chain with negative glomerular staining for (E) λ-light chain. Glomeruli were negative for IgG and IgA (not shown). Magnification, ×400 in A, D, and E; ×4800 in B; ×200 in C.
Figure 3.
Figure 3.
Lymphomatous infiltration of the kidney. Lymphomatous infiltration which can be seen alone or with other lesions in the kidney is common in patients with B-cell lymphoproliferative disorders. There is a dense patchy interstitial infiltration by monotonous small lymphocytes (hematoxylin and eosin). Immunohistochemical staining of this infiltrate was consistent with interstitial involvement by marginal zone lymphoma. Note the absence of tubulitis. Magnification, ×200.
Figure 4.
Figure 4.
(A) Median overall survival of 45 patients with monoclonal gammopathy–related kidney diseases from time of kidney biopsy. While both overall and kidney survival were inferior in patients with amyloid-related glomerulopathy, only the overall survival was statistically significant. Patients with amyloid-related glomerulopathy (n=19) are represented by the solid line, patients with nonamyloid-related glomerulopathy (n=18) are represented by the dashed line, and patients with tubulointerstitial nephropathies (n=8) are represented by the dotted line. Median survival was 64.4 months in patients with amyloid-related glomerulopathy and 160.5 months in the nonamyloid-related glomerulopathy group but had not been reached in patients with tubulointerstitial nephropathies (P=0.19). (B) Kidney survival from the time of kidney biopsy. Median kidney survival was reached only by patients with amyloid-related glomerulopathy (94.2 months; n=16), and it was not statistically different (P=0.19) from that of patients with nonamyloid-related glomerulopathy (n=16) or patients with tubulointerstitial nephropathies (n=7).

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