An Exceptional Case of Light Chain Only Variant of Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits Secondary to Chronic Lymphocytic Leukemia: A Case Report and Review of the Literature

We present the case of an 86-year-old Caucasian male with an 11-year history of low-grade chronic lymphocytic leukemia (CLL) presenting with nephrotic syndrome (NS). Renal biopsy findings showed a diffuse mesangial and endocapillary proliferative glomerulonephritis (GN) lesion with fine granular dep...

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Bibliographic Details
Published in:Case reports in nephrology 2022-10, Vol.2022, p.1-7
Main Authors: De La Flor, José C., Apaza, Jacqueline, Díaz, Francisco, Sandoval, Edna, Linares, Tania, Marschall, Alexander, Núñez, Patricia, Rivas-Nieto, Andrea Cecilia, Ruiz, Elisa
Format: Article
Language:English
Subjects:
Antibodies
Biopsy
Blood
Bone marrow
Case Report
Case reports
Creatinine
Disease
Edema
Immunoglobulins
Laboratories
Leukemia
Light
Lymphocytes
Microscopy
Proteins
Tomography
Urine
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Summary:We present the case of an 86-year-old Caucasian male with an 11-year history of low-grade chronic lymphocytic leukemia (CLL) presenting with nephrotic syndrome (NS). Renal biopsy findings showed a diffuse mesangial and endocapillary proliferative glomerulonephritis (GN) lesion with fine granular deposits, consistent with a rare morphologic variant of proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID)-lambda light chain (LC) only. Monthly combination therapy of rituximab (500 mg/m2 on day 1), fludarabine (30 mg/m2 on days 1–3), and cyclophosphamide (750 mg/m2 on days 1–3) was administered. Five courses of this regimen resulted in hematological remission, as well as a partial renal response with a reduction in the spot urine protein-to-creatinine ratio (UPCR) of 815.3 mg/g (reduction > 50% proteinuria without improvement in kidney function). This condition is a rare morphological variant of PGNMID, poorly described in CLL patients. We review the literature and suggest that this case provides sheds light on the unknown pathophysiological mechanisms of monoclonal immunoglobulins (MIg)-mediated glomerular damage in CLL patients, and may be helpful for the investigation of a more effective treatment.
ISSN:2090-6641
2090-665X
DOI:10.1155/2022/9207282