Unique Dystrophic Xanthomatous Reaction in Renal Parenchyma Due to T Cell Lymphoma

Introduction: While B-lymphocyte and plasma cell-associated kidney involvement have been described extensively in literature, kidney involvement by T-cell lymphoma remains a rare phenomenon. Rare case reports have described an interstitial nephritis-like picture in association with peripheral T cell lymphoma. We describe a unique dystrophic xanthomatous reaction in the kidney parenchyma in association with interstitial involvement with a CD8+ve T cell lymphoma. Case Description: A 23-year-old woman with autoimmune encephalitis, seizure disorder hypogammaglobulinemia due to Common Variable Immune Deficiency (CVID), and CD3+/CD8+ T cell lymphoproliferative involvement of the liver and bone marrow, was admitted for seizures, and found to have non-oliguric acute kidney injury (Creatinine (Cr) of 4.42 mg/dL (baseline 1) with severe metabolic acidosis (serum bicarbonate of 9 mmol/L). Urine showed hematuria and proteinuria of 1.6g/g. Kidney ultrasound showed bilateral enlarged kidneys measuring 14.8cm and 16cm with diffuse infiltrative process. Kidney biopsy showed unremarkable glomeruli and an abnormal-appearing tubulointerstitium in the form of diffuse interstitial sheets and intra-tubular collections of foam cells/histiocytes, along with a patchy infiltrate of atypical T-lymphoid cells of CD3/CD8-lineage. (image 1) She was started on methotrexate 5mg/m2 weekly and prednisone 1mg/kg by her oncologist. On follow up, kidney function had improved with most recent Cr of 1.98mg/dL. Discussion: We describe a unique histologic reaction (dystrophic xanthoma cells in the interstitium and within dilated kidney tubules) in association with interstitial involvement by a T-cell lymphoma, which has not been described before in the kidney, although such xanthomatous reaction has been described for cutaneous T-cell lymphomas. Awareness is vital in identifying such rare kidney histomorphology in association with T-cell lymphomas.

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Journal of the American Society of Nephrology
Published
2024-10-01
DOI
https://doi.org/10.1681/asn.202476ya0jxk
Primary Topic
Eosinophilic Disorders and Syndromes
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article
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article

Unique Dystrophic Xanthomatous Reaction in Renal Parenchyma Due to T Cell Lymphoma

Rajib Gupta, Sharmilee Vuyyuru, Nasim Wiegley, Hiba Hamdan
Journal of the American Society of Nephrology
Eosinophilic Disorders and Syndromes
article

Unique Dystrophic Xanthomatous Reaction in Renal Parenchyma Due to T Cell Lymphoma

Rajib Gupta, Sharmilee Vuyyuru, Nasim Wiegley, Hiba Hamdan
article en

Abstract

Introduction: While B-lymphocyte and plasma cell-associated kidney involvement have been described extensively in literature, kidney involvement by T-cell lymphoma remains a rare phenomenon. Rare case reports have described an interstitial nephritis-like picture in association with peripheral T cell lymphoma. We describe a unique dystrophic xanthomatous reaction in the kidney parenchyma in association with interstitial involvement with a CD8+ve T cell lymphoma. Case Description: A 23-year-old woman with autoimmune encephalitis, seizure disorder hypogammaglobulinemia due to Common Variable Immune Deficiency (CVID), and CD3+/CD8+ T cell lymphoproliferative involvement of the liver and bone marrow, was admitted for seizures, and found to have non-oliguric acute kidney injury (Creatinine (Cr) of 4.42 mg/dL (baseline 1) with severe metabolic acidosis (serum bicarbonate of 9 mmol/L). Urine showed hematuria and proteinuria of 1.6g/g. Kidney ultrasound showed bilateral enlarged kidneys measuring 14.8cm and 16cm with diffuse infiltrative process. Kidney biopsy showed unremarkable glomeruli and an abnormal-appearing tubulointerstitium in the form of diffuse interstitial sheets and intra-tubular collections of foam cells/histiocytes, along with a patchy infiltrate of atypical T-lymphoid cells of CD3/CD8-lineage. (image 1) She was started on methotrexate 5mg/m2 weekly and prednisone 1mg/kg by her oncologist. On follow up, kidney function had improved with most recent Cr of 1.98mg/dL. Discussion: We describe a unique histologic reaction (dystrophic xanthoma cells in the interstitium and within dilated kidney tubules) in association with interstitial involvement by a T-cell lymphoma, which has not been described before in the kidney, although such xanthomatous reaction has been described for cutaneous T-cell lymphomas. Awareness is vital in identifying such rare kidney histomorphology in association with T-cell lymphomas.

Journal of the American Society of NephrologyVol. 35(10S)
University of California, Davis (US)
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Openalex Percentile: Top 67%
Eosinophilic Disorders and Syndromes
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Unique Dystrophic Xanthomatous Reaction in Renal Parenchyma Due to T Cell Lymphoma — Rajib Gupta, Sharmilee Vuyyuru, et al. · Journal of the American Society of Nephrology (2024) | TGRS Research Map | TGRS