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Kidney Week

Abstract: FR-PO691

Intravascular Lymphoma of the Kidney and AKI

Session Information

Category: Trainee Case Report

  • 1500 Onco-Nephrology

Authors

  • Vasquez-Rios, George, Saint Louis University School of Medicine, Saint Louis, Missouri, United States
  • Wuliji, Natalia, Saint Louis University School of Medicine, Saint Louis, Missouri, United States
  • Shieh, Stephanie C., St. Louis VA Medical Center, Saint Louis, Missouri, United States
Introduction

Intravascular lymphoma of the kidney (ILK) is an extremely rare condition characterized by malignant infiltration of small vessels.

Case Description

A 66-year-old woman presented with fatigue, altered mental status, and acute onset diarrhea. On exam, she was pale with abdominal tenderness. CBC showed leukocytosis, anemia, and thrombocytopenia. Basic chemistry showed creatinine: 2.1 mg/dL (baseline: 0.8), BUN:15 mg/dL, Na:122 mmol/L, HCO3:17 mmol/L, markedly elevated LFTs, and lactic acid levels. Urinalysis showed: hematuria, mild proteinuria, and granular casts. Multiple studies including cancer work-up from blood, CSF, and BAL was only notable for high ESR. Pan CT scan revealed no lymphadenopathy. Bone marrow biopsy, flow cytometry, and genetic studies were also unmarkable. Despite the treatment with fluids and broad-spectrum antimicrobials, her clinical status continued to be poor with encephalopathy and acute kidney injury requiring dialysis. A kidney biopsy revealed heavy neoplastic cell infiltration of the renal cortex (nests surrounding renal tubules with marked interstitial hemorrhage) and prominent small vessel infiltration. Immunohistochemical staining showed that all malignant cells expressed CD45, PAX5, CD79a, CD20, BCL6, and MUM1, whereas staining for CD3, CD5, CD10, TdT, CD34, AE1/AE3, CAM 5.2, and MART1 was negative. Diagnosis of ILK was made. The patient was emergently treated with Rituximab, Cyclophosphamide, Etoposide, and steroids. Nevertheless, after a prolonged hospital course complicated by septic shock, and disseminated opportunistic infections, the patient expired.

Discussion

ILK is a devastating condition with a median survival of less than a year. Ante-mortem diagnosis is often missed due to the lack of detection of malignant cells in peripheral blood. Biopsy is mandatory whenever possible. Renal hemorrhage, and acute tubule-interstitial nephritis may be a presentation of this condition.