Characteristics of Renal Intravascular Large B-cell Lymphoma

Introduction: Renal intravascular large B-cell lymphoma (IVLBCL) is a rare, aggressive B-cell lymphoma with neoplastic cells occupying the vascular lumina with only 53 patients reported to date. Here, we present the largest case series to characterize this rare disease. Methods: We performed a multi-institutional, retrospective review of kidney biopsies and autopsies with a diagnosis of kidney IVLBCL and report our findings. Results: We identified 20 patients with an average age of 65.7 ± 7.8 years (55% males) with IVLBCL on kidney biopsy. The most common clinical presentation was fever and anemia. Acute kidney injury (AKI) was noted in 70% to 90%, proteinuria in 70% to 84.1%, hematuria in 45%, and nephrotic-range proteinuria in 10% to 26.1% of cases. The median (interquartile range) of serum creatinine was 1.75 (1.14, 3.3) mg/dl. Neoplastic lymphoid cells were present in glomeruli, peritubular capillaries, and arteries or veins. Of the patients, 44.3% showed extrarenal infiltration into bone marrow, liver, spleen, central vervous system, lung and skin. Neoplastic cells express CD20, CD79a, PAX-5, and MUM1+, and were CD10-negative. Available follow-up data showed a median survival of 21 months after diagnosis. Extrarenal involvement is a significant and independent predictor of mortality with a hazard ratio of 4.975 (95% confidence interval:1.38, 17.88) after controlling for age and gender. Serum creatinine, age, sex, and infiltration of intrarenal arteries or veins did not affect survival. Conclusion: Kidney IVLBCL is a rare disease that is unexpectedly diagnosed by kidney biopsy, presenting with fever, anemia, mild AKI, and proteinuria. Median survival is 21 months and extrarenal involvement is associated with worse outcome.