Division of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, 3-25-8 Nishi-Shimbashi, Minato-Ku, Tokyo, Japan.
CEN Case Rep. 2021 Feb;10(1):59-63. doi: 10.1007/s13730-020-00515-4. Epub 2020 Aug 9.
We report the first case of intravascular large B-cell lymphoma (IVLBCL) presenting with vasculitis-like symptoms and elevated serum levels of anti-neutrophil cytoplasmic antibody (ANCA) diagnosed by renal biopsy. The patient exhibited low-grade fever, systemic inflammatory reactions, multiple lung lesions, and persistent proteinuria, which were closely correlated with changes in serum titers of proteinase-3- and myeloperoxidase-ANCA. Preemptive therapy with prednisolone alone partially attenuated these symptoms. Renal biopsy did not reveal histopathological findings suggestive of granulomatous or microscopic polyangiitis. Glomerular and peritubular capillaries were diffusely occluded by CD20-positive large atypical mononuclear cells, with focal foot process effacement of podocytes in the glomeruli. Based on the specific immunophenotype of infiltrated atypical cells, the patient was diagnosed with IVLBCL. Chemotherapy regimens for IVLBCL improved clinical symptoms and led to remission of proteinuria. The ANCA titers decreased in parallel with reductions in the serum levels of the soluble interleukin-2 receptor, suggestive of an association between changes in ANCA levels and IVLBCL-related vascular injuries.
我们报告了首例以血管炎样症状和抗中性粒细胞胞质抗体（ANCA）升高为表现，经肾活检诊断的血管内大 B 细胞淋巴瘤（IVLBCL）。该患者表现为低热、全身炎症反应、多发肺部病变和持续性蛋白尿，与蛋白酶 3-和髓过氧化物酶-ANCA 血清滴度的变化密切相关。单独使用泼尼松龙的抢先治疗部分缓解了这些症状。肾活检未显示提示肉芽肿性或显微镜下多血管炎的组织病理学发现。肾小球和肾小管毛细血管被 CD20 阳性的大型异型单核细胞弥漫性阻塞，肾小球的足突细胞有局灶性足突融合。基于浸润性异型细胞的特定免疫表型，该患者被诊断为 IVLBCL。IVLBCL 的化疗方案改善了临床症状，并导致蛋白尿缓解。ANCA 滴度随可溶性白细胞介素-2 受体水平的降低而平行降低，提示 ANCA 水平的变化与 IVLBCL 相关的血管损伤之间存在关联。
