Renal Intravascular Large B-cell Lymphoma: A Case Report and Review of the Literature

Abstract

We herein report the case of a 52-year-old woman who consulted us because of a 2-month history of a fever, anorexia and weight loss. A physical examination was unremarkable. The blood count showed mild anemia and lymphopenia, and lactate dehydrogenase was elevated. Creatinine clearance was normal and proteinuria was undetectable. CT showed enlarged kidneys. A bone marrow biopsy was normal. PET-CT showed an intense uptake of 18fluorodeoxyglucose in both kidneys. A kidney biopsy provided the diagnosis of intravascular large B-cell lymphoma (IVLBCL). Kidney-limited IVLBCL without an impairment in the renal function or proteinuria has not been described. We analyzed the 38 published cases of IVLBCL involving the kidney to describe the main features of this entity.

Nephrotic syndrome without kidney injury revealing intravascular large B cell lymphoma

“…Pathogenesis of the nephrotic syndrome is not clear: minimal change disease induced by cytokines that increased glomerular membrane permeability, or invasion of glomeruli by lymphomatous cells leading to effacement of foot processes 8 10. Interestingly only a few cases of pure nephrotic syndrome without acute kidney injury or kidney enlargement caused by IVLBCL have been described in the literature, as now described in this case report 10–14…”

Intravascular Large B-Cell Lymphoma of the Kidney

“… 3 , 5 , 7 In kidney-proven cases of IVLBCL, renal failure, proteinuria, and fever have been described variably. 9 …”

“…In cases of IVLBCL involving the kidney, most present with proteinuria and some with nephrotic-range proteinuria. 9 The evidence of endothelial focal swelling and the formation of a new layer of basement membrane material under the endothelium (double contours, and without associated subendothelial electron-dense deposits) suggests acute and chronic endothelial injury; however, it is not entirely clear as to whether this process (in part or at all) is secondary to the lymphomatous cells in the microcirculation and/or another primary vascular disease present. Thrombotic angiopathies are associated with monoclonal Igs in the setting of a lymphoproliferative disorder or plasma cell neoplasm; however, our patient never had a paraprotein detected to which to ascribe the endothelial injury.…”

Limited Renal Intravascular Lymphoma: A Case Report and Review of the Literature

“…A diagnosis is made by conducting a surgical biopsy in the affected organ to determine if there has been infiltration by cancerous cells. Intravascular lymphoma (IVL) is positive in 91% of cases for B-cell phenotype (CD19, CD20, CD22, and CD79a) and has occasionally shown expression of CD5 with this last marker being associated with a poor prognosis [ 6 ]. Vieites et al and Yamamoto et al [ 7 , 8 ] described segmental tandem triplication of the 18q21 and q22q25 of chromosome 11 as genetic alterations of IVL.…”