Iwagami Masao, Furuya Rei, Tsutsumi Daimu, Mochida Yasuhiro, Ishioka Kunihiro, Oka Machiko, Maesato Kyoko, Moriya Hidekazu, Ohtake Takayasu, Hidaka Sumi, Kobayashi Shuzo
Department of Nephrology, Immunology, and Vascular Medicine, Shonan Kamakura General Hospital, 1370-1 Okamoto, Kamakura, Kanagawa, 247-8533, Japan.
CEN Case Rep. 2012 Nov;1(2):61-68. doi: 10.1007/s13730-012-0014-5. Epub 2012 May 26.
A 78-year-old Japanese female presented with low-grade fever, malaise, and appetite loss lasting for 1 month. Upper and lower gastrointestinal endoscopy and contrast-enhanced whole-body computed tomography (CT) revealed no abnormal findings at a referring hospital. She was referred to our hospital because of bilateral leg edema and 2.5 g/day proteinuria. Serum creatinine was 0.73 mg/dl and the kidneys were not enlarged. Kidney biopsy showed marked endocapillary proliferation with mesangiolysis. Soon after the kidney biopsy, her symptoms improved spontaneously, along with decreases in lactate dehydrogenase (LDH) from 503 to 197 IU/l, C-reactive protein (CRP) from 4.47 to 0.66 mg/dl, and soluble interleukin-2 receptor (sIL-2R) from 1789 to 1001 U/ml. Thus, she was followed carefully as an outpatient. One month later, however, she presented with dysarthria and right-sided hemiparesis, and diffusion-weighted brain magnetic resonance imaging (MRI) showed multiple high-intensity areas. She also had respiratory failure, and lung perfusion scintigraphy showed multiple low blood stream areas. Suspecting some endovascular abnormality, we performed immunohistochemical staining of the kidney biopsy specimen taken previously to find that endocapillary infiltrating cells were CD20-positive B lymphocytes. The infiltrating cells were confined to the endocapillary compartment in glomeruli and peritubular capillaries. Both clinical and pathological findings led us to diagnose intravascular large B cell lymphoma (IVLBCL). Two bone marrow biopsies and random skin biopsies were performed, but no abnormality was found. The present case demonstrates that clinical course and renal biopsy findings of intravascular large B cell lymphoma may mimic other renal conditions and that the identification of cell types with immunohistochemical staining may help establish an accurate diagnosis.
一名78岁的日本女性出现低热、乏力和食欲减退症状，持续1个月。转诊医院的上下消化道内镜检查和增强全身计算机断层扫描（CT）未发现异常。因双侧腿部水肿和每日2.5克蛋白尿，她被转诊至我院。血清肌酐为0.73毫克/分升，肾脏未增大。肾活检显示明显的毛细血管内增生伴血管系膜溶解。肾活检后不久，她的症状自发改善，同时乳酸脱氢酶（LDH）从503降至197国际单位/升，C反应蛋白（CRP）从4.47降至0.66毫克/分升，可溶性白细胞介素-2受体（sIL-2R）从1789降至1001单位/毫升。因此，她作为门诊患者接受密切随访。然而，1个月后，她出现构音障碍和右侧偏瘫，弥散加权脑磁共振成像（MRI）显示多个高强度区域。她还出现呼吸衰竭，肺灌注闪烁显像显示多个低血流区域。怀疑存在一些血管内异常，我们对之前采集的肾活检标本进行免疫组织化学染色，发现毛细血管内浸润细胞为CD20阳性B淋巴细胞。浸润细胞局限于肾小球和肾小管周围毛细血管的毛细血管内区域。临床和病理结果均使我们诊断为血管内大B细胞淋巴瘤（IVLBCL）。进行了两次骨髓活检和随机皮肤活检，但未发现异常。本病例表明，血管内大B细胞淋巴瘤的临床病程和肾活检结果可能与其他肾脏疾病相似，免疫组织化学染色鉴定细胞类型有助于准确诊断。
