Monotypic plasma cell interstitial nephritis as the only clinical manifestation in a patient with previously undiagnosed indolent multiple myeloma: A case report.

Medicine (Baltimore)

Assistance Publique-Hôpitaux de Paris (AP-HP), Service de Néphrologie et Transplantation, Institut Francilien de Recherche en Néphrologie et Transplantation (IFRNT), Centre de Référence Maladie Rare Syndrome Néphrotique Idiopathique, Groupe Hospitalier Henri-Mondor/Albert-Chenevier AP-HP, Département de Pathologie, Groupe Hospitalier Henri-Mondor/Albert-Chenevier Université Paris-Est-Créteil (UPEC), Département Hospitalo-Universitaire (DHU) Virus-Immunité-Cancer (VIC), Institut Mondor de Recherche Biomédicale (IMRB), Equipe 21, INSERM U 955 AP-HP, Unité d'Hémopathies Lymphoïdes, Groupe Hospitalier Henri-Mondor/Albert-Chenevier UPEC, DHU VIC, IMRB, Equipe 9, INSERM U 955, Créteil Centre de Référence des Amyloses Primitives et des Maladies de Dépôts d'Immunoglobulines Monoclonales Département de Pathologie, Centre Hospitalier Universitaire de Poitiers, Poitiers, France.

Published: August 2016

Introduction: Predominantly monotypic plasma cell infiltrates are an uncommon renal finding in patients with malignant lymphoplasmacytic proliferation.

Case Presentation: We report the case of a 52-year-old man with chronic kidney disease and significant proteinuria associated with a monoclonal immunoglobulin spike (IgGκ). Kidney biopsy revealed the presence of atypical multinucleated CD138 plasma cells with voluminous nuclei stained exclusively with a κ antibody. Electron microscopy showed mesangial and segmental parietal electron-dense, nonorganized hyaline deposits without immunogold labeling for the κ light chain. The bone marrow aspirate revealed 6% of apparently mature plasmocytes without dystrophy. We therefore concluded that the patient had an indolent multiple myeloma with specific renal involvement in the form of malignant monotypic interstitial plasmacytic infiltration. We initiated a specific chemotherapy regimen including bortezomib-cyclophosphamide-dexamethasone. After 4 months of follow-up, creatinine levels had improved slightly and free κ light-chain levels had decreased significantly within the normal range.

Conclusion: This case highlights the need to consider neoplastic interstitial plasma cell infiltration systematically in patients diagnosed with an apparently benign monoclonal gammopathy and to consider adaptation of the chemotherapy regimen, to improve renal function.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4979806PMC
http://dx.doi.org/10.1097/MD.0000000000004391DOI Listing

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