Journal article
Characteristics of Renal Intravascular large B-cell Lymphoma
Kidney international reports, Vol.8(3), pp.575-583
03/2023
DOI: 10.1016/j.ekir.2022.12.001
PMCID: PMC10014346
PMID: 36938088
Abstract
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.
We performed a multi-institutional, retrospective review of kidney biopsies and autopsies with a diagnosis of kidney IVLBCL and report our findings.
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 70-90%, proteinuria in 70-84.1%, hematuria in 45%, and nephrotic range proteinuria in 10-26.1% 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. 44.3% of patients showed extrarenal infiltration into bone marrow, liver, spleen, CNS, 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% CI:1.38, 17.88) after controlling for age and gender. Serum creatinine, age, sex, and infiltration of intra-renal arteries or veins did not affect survival.
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.
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Details
- Title: Subtitle
- Characteristics of Renal Intravascular large B-cell Lymphoma
- Creators
- Prerna Rastogi - University of IowaAudai Alrwashdeh - Department of Pathology, University of Iowa Hospitals and Clinics, Iowa City, IATiffany Caza - Arkana LaboratoriesMercury Lin - Department of Pathology and Laboratory Medicine, Cedars Sinai, Los Angeles, CAMohammad Obeidat - University of IowaGabriel Giannini - Arkana LaboratoriesChris Larsen - Arkana LaboratoriesDao-Fu Dai - Department of Pathology, University of Iowa Hospitals and Clinics, Iowa City, IA
- Resource Type
- Journal article
- Publication Details
- Kidney international reports, Vol.8(3), pp.575-583
- Publisher
- Elsevier Inc
- DOI
- 10.1016/j.ekir.2022.12.001
- PMID
- 36938088
- PMCID
- PMC10014346
- ISSN
- 2468-0249
- eISSN
- 2468-0249
- Grant note
- DOI: 10.13039/100000002, name: National Institutes of Health, award: HL145138; DOI: 10.13039/100008893, name: The University of Iowa, award: DK133118
- Language
- English
- Electronic publication date
- 12/2022
- Date published
- 03/2023
- Academic Unit
- Pathology; Iowa Neuroscience Institute; Radiation Oncology
- Record Identifier
- 9984339312602771
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