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免疫母细胞性 T 细胞淋巴瘤患者的急进性肾小球肾炎:一例罕见尸检病例表现为 IgA 血管炎和管状沉积。

Rapidly progressive glomerulonephritis in a patient with angioimmunoblastic T-cell lymphoma: a rare autopsy case showing IgA vasculitis and cylinder-like deposits.

机构信息

Department of Pathology, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, 980-8574, Japan.

Department of Hematology and Rheumatology, Tohoku University Graduate School, Miyagi, Japan.

出版信息

Med Mol Morphol. 2022 Sep;55(3):267-273. doi: 10.1007/s00795-022-00325-w. Epub 2022 Jun 3.

Abstract

Angioimmunoblastic T-cell lymphoma (AITL), a hematological malignancy, originates from follicular helper T cells. The primary site of AITL is the lymph nodes, but extranodal presentation is frequent in patients with advanced stages. Here, we report a rare case of a patient with AITL presenting with rapidly progressive glomerulonephritis (RPGN). The patient underwent computed tomography, which showed systemic lymph node swelling. RPGN was noted at the time of admission. Livedo was observed in the lower limbs with purpura on the foot. The patient was diagnosed with AITL based on lymph node biopsy. Skin biopsy revealed vasculitis with immunoglobulin A (IgA) deposits. Renal biopsy revealed endocapillary proliferative glomerulonephritis with massive subendothelial deposits and intraluminal thrombi. Immunofluorescence showed IgA, IgG, and complement component 3c-predominant granular staining pattern in the capillary and mesangial areas. Electron micrographs demonstrated dense cylindrical-like deposits in the subendothelial space. Chemotherapy drugs were administered, but the patient's respiratory distress increased until death. Upon autopsy, membranoproliferative glomerulonephritis and extensive necrotizing cellular crescent formation were observed in the glomeruli. Taken together, this case is a rare combination of AITL and RPGN showing both cylinder-like deposits suggestive of cryoglobulinemic glomerulonephritis (CN) and IgA vasculitis.

摘要

血管免疫母细胞性 T 细胞淋巴瘤(AITL)是一种血液系统恶性肿瘤,起源于滤泡辅助 T 细胞。AITL 的主要部位是淋巴结,但在晚期患者中常出现结外表现。在这里,我们报告了一例罕见的 AITL 患者表现为快速进行性肾小球肾炎(RPGN)。患者行计算机断层扫描,显示全身淋巴结肿大。入院时发现 RPGN。下肢有淤点性红斑伴皮肤紫斑。根据淋巴结活检诊断为 AITL。皮肤活检显示免疫球蛋白 A(IgA)沉积的血管炎。肾活检显示内皮下增生性肾小球肾炎,大量内皮下沉积物和管腔内血栓形成。免疫荧光显示毛细血管和系膜区 IgA、IgG 和补体成分 3c 为主的颗粒状染色模式。电子显微镜显示在下皮间隙有致密的柱状样沉积物。给予化疗药物,但患者的呼吸困难逐渐加重,直至死亡。尸检时,肾小球观察到膜增殖性肾小球肾炎和广泛的坏死性细胞新月体形成。综上所述,本例为 AITL 和 RPGN 的罕见组合,既有提示冷球蛋白血症性肾小球肾炎(CN)的柱状沉积,也有 IgA 血管炎。

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