Cast nephropathy and light-chain deposition disease in Waldenström macroglobulinemia.
Waldenström macroglobulinemia is a rare low-grade hematologic malignancy due to clonal proliferation of B lymphocytes responsible for immunoglobulin M (IgM) monoclonal gammopathy secreted in serum. This disease is characterized by lymphoplasmacytic tumoral infiltration of bone marrow and various organs, especially the liver and spleen. Kidney involvement in Waldenström macroglobulinemia has been described previously with reports of various forms of glomerular injury: large intracapillary IgM pseudothrombi, cryoglobulinemia-associated membranoproliferative glomerulonephritis, or amyloidosis. Interstitial infiltration by tumoral B lymphocytes is another classic pattern. Conversely, tubular involvement in the form of myeloma-like casts or basement membrane deposition of monoclonal light chain (light-chain deposition disease) is unusual. We report the occurrence of cast nephropathy associated with light-chain deposition disease in 2 patients with Waldenström macroglobulinemia, which resulted in severe and prolonged kidney failure.
Gnemmi V
，Leleu X
，Provot F
，Moulonguet F
，Buob D
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Kidney diseases associated with monoclonal immunoglobulin M-secreting B-cell lymphoproliferative disorders: a case series of 35 patients.
Kidney diseases associated with immunoglobulin M (IgM) monoclonal gammopathy are poorly described, with few data for patient outcomes and renal response.
Case series.
35 patients from 8 French departments of nephrology were retrospectively studied. Inclusion criteria were: (1) detectable serum monoclonal IgM, (2) estimated glomerular filtration rate (eGFR) < 60mL/min/1.73m(2) and/or proteinuria with protein excretion > 0.5g/d and/or microscopic hematuria, and (3) kidney biopsy showing monoclonal immunoglobulin deposits and/or lymphomatous B-cell renal infiltration. All patients received chemotherapy, including rituximab-based regimens in 8 cases.
Patients were classified into 3 groups according to renal pathology: glomerular AL amyloidosis (group 1; n=11), nonamyloid glomerulopathies (group 2; n=15, including 9 patients with membranoproliferative glomerulonephritis), and tubulointerstitial nephropathies (group 3; n=9, including cast nephropathy in 5, light-chain Fanconi syndrome in 3, and isolated tumor infiltration in 1).
Posttreatment hematologic response (≥50% reduction in serum monoclonal IgM and/or free light chain level) and renal response (≥50% reduction in 24-hour proteinuria or eGFR≥30mL/min/1.73m(2) in patients with glomerular and tubulointerstitial disorders, respectively).
Nephrotic syndrome was observed in 11 and 6 patients in groups 1 and 2, respectively. Patients in group 3 presented with acute kidney injury (n=7) and/or proximal tubular dysfunction (n=3). Waldenström macroglobulinemia was present in 26 patients (8, 12, and 6 in groups 1, 2, and 3, respectively). Significant lymphomatous interstitial infiltration was observed in 18 patients (4, 9, and 5 patients, respectively). Only 9 of 29 evaluable patients had systemic signs of symptomatic hematologic disease (2, 5, and 2, respectively). In groups 1, 2, and 3, respectively, hematologic response was achieved after first-line treatment in 3 of 9, 9 of 10, and 5 of 6 evaluable patients, while renal response occurred in 5 of 10, 9 of 15, and 5 of 8 evaluable patients.
Retrospective study; insufficient population to establish the impact of chemotherapy.
IgM monoclonal gammopathy is associated with a wide spectrum of renal manifestations, with an under-recognized frequency of tubulointerstitial disorders.
Chauvet S
，Bridoux F
，Ecotière L
，Javaugue V
，Sirac C
，Arnulf B
，Thierry A
，Quellard N
，Milin S
，Bender S
，Goujon JM
，Jaccard A
，Fermand JP
，Touchard G
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Kidney Involvement of Patients with Waldenström Macroglobulinemia and Other IgM-Producing B Cell Lymphoproliferative Disorders.
Kidney involvement in Waldenström macroglobulinemia is less well described compared with kidney manifestations in multiple myeloma.
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.
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.
Our study confirms a diverse variety of kidney lesions in patients with monoclonal IgM gammopathy.
Higgins L
，Nasr SH
，Said SM
，Kapoor P
，Dingli D
，King RL
，Rajkumar SV
，Kyle RA
，Kourelis T
，Gertz MA
，Dispenzieri A
，Lacy MQ
，Buadi FK
，Ansell SM
，Gonsalves WI
，Thompson CA
，Fervenza FC
，Zand L
，Hwa YL
，Jevremovic D
，Shi M
，Leung N
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