Nasr Samih H, Shanafelt Tait D, Hanson Curtis A, Fidler Mary E, Cornell Lynn D, Sethi Sanjeev, Chaffee Kari G, Morris Joseph, Leung Nelson
Division of Anatomic Pathology, Mayo Clinic, Rochester, MN.
Division of Hematology, Mayo Clinic, Rochester, MN.
Ann Diagn Pathol. 2015 Jun;19(3):130-6. doi: 10.1016/j.anndiagpath.2015.03.003. Epub 2015 Mar 6.
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.
肉芽肿性间质性肾炎（GIN）是一种罕见的病理病变，在0.5%至5.9%的肾活检中可见。药物、结节病和感染是大多数GIN病例的病因。恶性肿瘤并非GIN的既定病因。在此，我们报告了一系列5例继发于慢性淋巴细胞白血病/小淋巴细胞淋巴瘤（CLL/SLL）的GIN患者。患者大多为老年白人男性，有CLL/SLL病史，表现为严重肾功能损害（血清肌酐峰值中位数为7.3mg/dL）、白细胞尿和轻度蛋白尿。1例有肾肿大。2例患者肾功能不全的发生和复发与淋巴细胞增多水平密切相关。所有患者的肾活检均显示GIN伴CLL/SLL白血病性间质浸润。肉芽肿为非坏死性上皮样，伴有巨细胞。1例活检显示肉芽肿性动脉炎。1例患者在淋巴结和皮肤有肉芽肿反应。使用/不使用针对CLL/SLL的化疗的类固醇治疗使除1例活检显示有晚期皮质瘢痕形成的患者外的所有患者肾功能部分改善。总之，我们报告了CLL/SLL与GIN之间的关联。患者通常因GIN和白血病性间质浸润而出现严重肾衰竭，这往往对使用/不使用针对CLL/SLL的化疗的类固醇治疗有反应。这种临床情况下GIN的发病机制尚不清楚，但可能代表对CLL/SLL肿瘤细胞的局部超敏反应。
