血管免疫母细胞性T细胞淋巴瘤患者伴束状纤维化的肾小管间质性肾炎
Tubulointerstitial nephritis with storiform fibrosis in a patient with angioimmunoblastic T-cell lymphoma.
作者信息
Fukuda Haruka, Kitamura Mineaki, Sawase Atsushi, Uramatsu Tadashi, Yamashita Hiroshi, Tsushima Hideki, Irie Junji, Katafuchi Eisuke, Kato Seiya, Takeuchi Mai, Ohshima Koichi, Nakayama Toshiyuki, Mukae Hiroshi, Nishino Tomoya
机构信息
Department of Nephrology, Nagasaki University Graduate School of Biomedical Sciences, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan.
Department of Nephrology, Nagasaki Harbor Medical Center, Nagasaki, Japan.
出版信息
CEN Case Rep. 2025 Feb;14(1):39-46. doi: 10.1007/s13730-024-00901-2. Epub 2024 Jun 20.
We present a case of an angioimmunoblastic T-cell lymphoma (AITL) and tubulointerstitial nephritis with storiform fibrosis in a 76-year-old man. The patient exhibited lymphadenopathy, polyclonal hypergammaglobulinemia, and renal dysfunction and was diagnosed with AITL on the basis of lymph node biopsy findings. The serum IgG4 level was highly elevated. Renal biopsy revealed IgG4-positive plasma cells and storiform fibrosis without infiltration of AITL, and the findings indicated IgG4-related kidney disease (IgG4-RKD). Following THPCOP therapy for AITL, the renal function improved. While diagnosing IgG4-RKD in a patient with AITL poses challenges, follicular helper T cell involvement appeared crucial in AITL and renal tubulointerstitial lesions in this case.
我们报告一例76岁男性血管免疫母细胞性T细胞淋巴瘤（AITL）合并伴束状纤维化的肾小管间质性肾炎。患者表现为淋巴结病、多克隆高球蛋白血症和肾功能不全，根据淋巴结活检结果诊断为AITL。血清IgG4水平显著升高。肾活检显示IgG4阳性浆细胞和束状纤维化，无AITL浸润，这些发现提示IgG4相关性肾病（IgG4-RKD）。针对AITL进行THPCOP治疗后，肾功能有所改善。虽然在AITL患者中诊断IgG4-RKD具有挑战性，但在该病例中，滤泡辅助性T细胞的参与似乎在AITL和肾小管间质病变中起关键作用。
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