Pathology of the Kidney in Waldenström's Macroglobulinemia

Morel-Marogér, L; Basch, A.; Danon, F; Verroust, P; Richet, G

doi:10.1056/nejm197007162830304

Cited by 142 publications

(76 citation statements)

References 18 publications

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“…No EM was performed. All patients with intraglomerular thrombi or deposits were found to have either a cryoglobulin or a high level of circulating IgM, which led Morel-Maroger et al (3) to suggest that the circulating monoclonal IgM might precipitate in the capillary lumens because of increased blood viscosity. Several isolated cases of ICMDD have been reported since (18 -20).…”

Section: Discussionmentioning

confidence: 99%

“…At the time of renal disease diagnosis, the lymphoproliferative disorder consisted of WM in seven patients (1,2,3,7,8,9, and 11), small B cell non-Hodgkin lymphoma in two patients (4 and 12), multiple myeloma in one patient (patient 5), and B cell lymphoma of the splenic marginal zone in one patient (patient 10). Three patients (6, 13, and 14) had an "IgM-related disorder.…”

Section: Hematologic and Immunologic Datamentioning

confidence: 99%

“…Their incidence seems to have decreased, mostly because of improved therapy of Waldenströ m macroglobulinemia (WM), the major cause of those nephropathies. First reviewed by Morel-Maroger et al (3), WM-related nephropathies include characteristic intracapillary deposits of IgM with or without cryoglobulinemia, AL-amyloidosis, and infiltration of the interstitium by neoplastic lymphoplasmacytic cells. Subsequent case reports included immunotactoid and nonamyloid fibrillary glomerulopathy (4,5), cryoglobulinemia-related glomerulonephritis (6), and crescentic glomerulonephritis (7,8).…”

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confidence: 99%

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Renal Lesions Associated with IgM-Secreting Monoclonal Proliferations

Audard¹,

Georges²,

Vanhille³

et al. 2008

Clinical Journal of the American Society of Nephrology

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Section: Discussionmentioning

confidence: 99%

Section: Hematologic and Immunologic Datamentioning

confidence: 99%

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confidence: 99%

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Renal Lesions Associated with IgM-Secreting Monoclonal Proliferations

Audard¹,

Georges²,

Vanhille³

et al. 2008

Clinical Journal of the American Society of Nephrology

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“…33 Renal failure is not very common; however, monoclonal IgM may accumulate in the renal glomeruli, forming subendothelial deposits that clog glomerular capillaries. 34 In this case, there may be moderate but reversible proteinuria, being the majority of patients asymptomatic.…”

Section: Clinical Symptomsmentioning

confidence: 93%

Waldenström's macroglobulinemia - a review

Coimbra

Neves

Lima

et al. 2014

Rev. Assoc. Med. Bras.

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Waldenström's macroglobulinemia (WM) is a lymphoproliferative disease of B lymphocytes, characterized by a lymphoplasmocytic lymphoma in the bone marrow and by IgM monoclonal hypergammaglobulinemia. It was first described in 1944 by Jan Gösta Waldenström, reporting two patients with oronasal bleeding, lymphadenopathy, anemia, thrombocytopenia, high erythrocyte sedimentation rate and serum viscosity, normal radiography and bone marrow infiltrated by lymphoid cells. The WM is a rare disease with a typically indolent clinical course, affecting mainly individuals aged between 63 and 68 years. Most patients have clinical signs and symptoms related to hyperviscosity resulting from IgM monoclonal gammopathy, and/or cytopenias resulting from bone marrow infiltration by lymphoma. The differential diagnosis with other lymphomas is essential for the assessment of prognosis and therapeutic approach. Treatment of patients with asymptomatic WM does not improve the quality of life of patients, or increase their survival, being recommended, therefore, their follow-up. For the treatment of symptomatic patients, alkylating agents, purine analogs and anti-CD20 monoclonal antibodies are used. However, the disease is incurable and the response to therapy is not always favorable. Recent studies have shown promising results with bortezomib, an inhibitor of proteasomes, and some patients respond to thalidomide. In patients with relapse or refractory to therapy, autologous transplantation may be indicated. The aim of this paper is to describe in detail the current knowledge on the pathophysiology of WM, main clinical manifestations, diagnosis, prognosis and treatment.

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“…Patients with less lymphoplasmocytic lymphoma involvement are considered to have IgM MGUS, and those with .10% but no end organ damage are classified as smoldering Waldenström macroglobulinemia (67). In the past, dense eosinophilic occlusive capillary thrombi were commonly found (68). All patients with these endocapillary thrombi had either cryoglobulins or high concentrations of monoclonal IgM.…”

Section: Lymphoplasmocytic Lymphoma/waldenströ M Macroglobulinemiamentioning

confidence: 99%

Dysproteinemias and Glomerular Disease

Leung

Drosou

Nasr

2017

CJASN

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Dysproteinemia is characterized by the overproduction of an Ig by clonal expansion of cells from the B cell lineage. The resultant monoclonal protein can be composed of the entire Ig or its components. Monoclonal proteins are increasingly recognized as a contributor to kidney disease. They can cause injury in all areas of the kidney, including the glomerular, tubular, and vascular compartments. In the glomerulus, the major mechanism of injury is deposition. Examples of this include Ig amyloidosis, monoclonal Ig deposition disease, immunotactoid glomerulopathy, and cryoglobulinemic GN specifically from types 1 and 2 cryoglobulins. Mechanisms that do not involve Ig deposition include the activation of the complement system, which causes complement deposition in C3 glomerulopathy, and cytokines/growth factors as seen in thrombotic microangiopathy and precipitation, which is involved with cryoglobulinemia. It is important to recognize that nephrotoxic monoclonal proteins can be produced by clones from any of the B cell lineages and that a malignant state is not required for the development of kidney disease. The nephrotoxic clones that do not meet requirement for a malignant condition are now called monoclonal gammopathy of renal significance. Whether it is a malignancy or monoclonal gammopathy of renal significance, preservation of renal function requires substantial reduction of the monoclonal protein. With better understanding of the pathogenesis, clone-directed strategies, such as rituximab against CD20 expressing B cell and bortezomib against plasma cell clones, have been used in the treatment of these diseases. These clone-directed therapies been found to be more effective than immunosuppressive regimens used in nonmonoclonal protein-related kidney diseases.

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Pathology of the Kidney in Waldenström's Macroglobulinemia

Cited by 142 publications

References 18 publications

Renal Lesions Associated with IgM-Secreting Monoclonal Proliferations

Renal Lesions Associated with IgM-Secreting Monoclonal Proliferations

Waldenström's macroglobulinemia - a review

Dysproteinemias and Glomerular Disease

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