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Abstract: TH-PO585

Renal Involvement in Sarcoidosis-Lymphoma Syndrome: A Report of Two Cases

Session Information

  • Pathology and Lab Medicine
    November 03, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
    Abstract Time: 10:00 AM - 12:00 PM

Category: Pathology and Lab Medicine

  • 1700 Pathology and Lab Medicine

Authors

  • Dzedzik, Siarhei, New York-Presbyterian Hospital, New York, New York, United States
  • Glezerman, Ilya, Memorial Sloan Kettering Cancer Center, New York, New York, United States
  • Park, Hye-Ran, Kidney Care Specialists-Medical Associates of Drexel Hill, Drexel Hill, Pennsylvania, United States
  • Salvatore, Steven, Weill Cornell Medicine, New York, New York, United States
  • Seshan, Surya V., Weill Cornell Medicine, New York, New York, United States
Introduction

Sarcoidosis-lymphoma syndrome (S-LS) is a condition characterized by the development of sarcoidosis and lymphoma in the same patient. Chronic inflammation is a putative mediator of this risk. Documented locations affected by sarcoidosis are lymph nodes, lung, bone marrow and spleen. Clinically significant renal involvement by sarcoidosis has not been reported in S-LS.

Case Description

Patient #1: 52-year-old male with small lymphocytic lymphoma (SLL) treated with steroids and rituximab (2018), sarcoidosis (2018) and chronic kidney disease (2019). In November 2021 he developed acute kidney injury, refractory hypercalcemia and lung nodules. Complete blood count (CBC) - hemoglobin 8.8 g/dL, WBC 6.2. Serum findings - creatinine 4.5 mg/dL (baseline 2.1 mg/dL), glucose 103 mg/dL, albumin 3.4 g/dL, calcium 13.2 mg/dL, ACE level 71 (normal <40).
Patient #2: 58-year-old female with TP53-mutated chronic lymphocytic leukemia (CLL) treated with fludarabine, bendamustine and rituximab (2002). In 2019 patient started ibrutinib with good response. One year later she developed anterior uveitis, lymphadenopathy, fever and night sweats. Lymph node biopsy confirmed sarcoidosis. Steroid course was initiated with resolution of the symptoms. In November 2021, one month before kidney biopsy, PET-CT showed CLL progression. CBC - hemoglobin 13.2 g/dL, WBC 4.77. Serum findings - creatinine 1.9 mg/dL (baseline 1.0 mg/dL), glucose 67 mg/dL, albumin 3.9 g/dL, calcium 9.4 mg/dL, ACE level 42.
In both cases there was no hematuria or proteinuria on urinalysis. Kidney biopsies demonstrated diffuse interstitial nephritis with non-caseating granulomatous inflammation and widespread tubular atrophy with interstitial fibrosis suggestive of sarcoidosis. No other infections or medications could be implicated. No evidence of SLL/CLL was identified.

Discussion

The differential diagnosis of impaired kidney function in patients with hematologic malignancies is broad. Infections, drug toxicity and direct or indirect involvement by the malignancy should be considered. Renal biopsy remains a definitive modality for an accurate diagnosis. These cases represent the first reported description of renal sarcoidosis as a part of S-LS. Renal pathologists and nephrologists should be aware of this association for potential accurate diagnosis of both entities and appropriate treatment.