title: Granulomatous interstitial nephritis secondary to chronic lymphocytic leukemia/small lymphocytic lymphoma.
abstract: Granulomatous interstitial nephritis (GIN) is an uncommon pathologic lesion encountered in 0.5% to 5.9% of renal biopsies. Drugs, sarcoidosis, and infections are responsible for most cases of GIN. Malignancy is not an established cause of GIN. Here, we report a series of 5 patients with GIN secondary to chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). Patients were mostly elderly white males with an established history of CLL/SLL who presented with severe renal impairment (median peak serum creatinine, 7.3 mg/dL), leukocyturia, and mild proteinuria. One had nephromegaly. In 2 patients, the development and relapse of renal insufficiency closely paralleled the level of lymphocytosis. Kidney biopsy in all patients showed GIN concomitant with CLL/SLL leukemic interstitial infiltration. Granulomas were nonnecrotizing and epithelioid and were associated with giant cells. One biopsy showed granulomatous arteritis. One patient had a granulomatous reaction in lymph nodes and skin. Steroids with/without CLL/SLL-directed chemotherapy led to partial improvement of kidney function in all patients except 1 who had advanced cortical scarring on biopsy. In conclusion, we report an association between CLL/SLL and GIN. Patients typically present with severe renal failure due to both GIN and leukemic interstitial infiltration, which tends to respond to steroids with/without CLL/SLL-directed chemotherapy. The pathogenesis of GIN in this clinical setting is unknown but may represent a local hypersensitivity reaction to the CLL/SLL tumor cells.
authors: Division of Anatomic Pathology, Mayo Clinic, Rochester, MN. Electronic address: nasr.samih@mayo.edu.
authors: Samih H
authors: SH
authors: Nasr
authors: Division of Hematology, Mayo Clinic, Rochester, MN.
authors: Tait D
authors: TD
authors: Shanafelt
authors: Division of Hematopathology, Mayo Clinic, Rochester, MN.
authors: Curtis A
authors: CA
authors: Hanson
authors: Division of Anatomic Pathology, Mayo Clinic, Rochester, MN.
authors: Mary E
authors: ME
authors: Fidler
authors: Division of Anatomic Pathology, Mayo Clinic, Rochester, MN.
authors: Lynn D
authors: LD
authors: Cornell
authors: Division of Anatomic Pathology, Mayo Clinic, Rochester, MN.
authors: Sanjeev
authors: S
authors: Sethi
authors: Department of Health Sciences Research, Mayo Clinic, Rochester, MN.
authors: Kari G
authors: KG
authors: Chaffee
authors: Great River Nephrology, West Burlington, IA.
authors: Joseph
authors: J
authors: Morris
authors: Division of Hematology, Mayo Clinic, Rochester, MN; Division of Nephrology, Mayo Clinic, Rochester, MN.
authors: Nelson
authors: N
authors: Leung
mesh_terms: D000368:Aged; D001706:Biopsy; D003937:Diagnosis, Differential; D005260:Female; D006099:Granuloma / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009395:Nephritis, Interstitial / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology*; D012008:Recurrence
keywords: Granulomatous interstitial nephritis; chronic lymphocytic leukemia; interstitial nephritis; kidney biopsy; lymphoma; small lymphocytic lymphoma