背景:TAFRO综合征是特发性多中心Castleman病(iMCD)的临床亚型,其特征是血小板减少症,Anasarca,发热和/或血清C反应蛋白升高,肾功能不全,和器官肿大。病例介绍:一名28岁的发烧妇女,体重增加13公斤,下肢水肿,肝脾肿大,多中心周围淋巴结肿大转诊至我们中心.实验室检查显示贫血,血小板减少症,肌酐为1.19mg/dL,低蛋白血症为33g/L。蛋白尿以2g/天测量,包括白蛋白尿以1.5g/天。尿沉渣检查发现白细胞尿症为44,000/mL,血尿为645,000/mL。血管内皮生长因子(VEGF)水平升高。颈淋巴结活检发现与iMCD混合组织病理学亚型一致的特征。肾活检显示膜增生性肾小球肾炎(MPGN)模式。我们开始了为期3天的甲基强的松龙脉冲治疗,每天1,000毫克,其次是泼尼松1mg/kg/天,进化有利。文献复习:19例iMCD合并TAFRO综合征患者行肾活检:作者诊断符合MPGN样8例,血栓性微血管病(TMA)样肾小球病11例,肾小球毛细血管或小动脉无纤维蛋白血栓,无典型生物学征象。临床,生物,在描述为具有MPGN和TMA样表现的病例之间,结局特征相似。在对每个病例的组织病理学描述进行彻底审查后,MPGN病变似乎是持续性TMA中慢性肾小球内皮损伤的后果。我们怀疑VEGF和IL-6在TAFRO综合征中观察到的从TMA样到MPGN的肾脏受累谱的生理病理学中起关键作用。结论:我们介绍了一名高加索iMCD患者,患有TAFRO综合征并继发于MPGN的肾功能不全,可能继发于慢性TMA样疾病。我们怀疑VEGF和IL-6对TAFRO综合征的TMA和MPGN病变之间存在连续性。
Background: TAFRO syndrome is a clinical subtype of idiopathic multicentric Castleman disease (iMCD) that is characterized by thrombocytopenia, anasarca, fever and/or elevated serum C-reactive protein, renal dysfunction, and organomegaly. Case Presentation: A 28-year-old woman with fever, weight gain of 13 kgs, lower extremity edema, hepatosplenomegaly, and multicentric peripheral lymphadenopathy was referred to our center. Laboratory investigations revealed anemia, thrombocytopenia, creatinine at 1.19 mg/dL and hypoalbuminemia at 33 g/L. Proteinuria was measured at 2 g/day including albuminuria at 1.5 g/day. Urinary sediment examination found leukocyturia at 44,000/mL and hematuria at 645,000/mL. Vascular endothelial growth factor (VEGF) level was elevated. A cervical lymph node biopsy found features consistent with the mixed histopathological subtype of iMCD. A renal biopsy revealed a membranoproliferative glomerulonephritis (MPGN) pattern. We initiated 3 days of methylprednisolone pulse-therapy at 1,000 mg per day, followed by prednisone 1 mg/kg/day and evolution was favorable.
Review of Literature: 19 iMCD patients with TAFRO syndrome had undergone a renal biopsy: 8 cases with author\'s diagnosis consistent with MPGN-like and 11 cases of thrombotic microangiopathy (TMA)-like glomerulopathy without fibrin thrombi in glomerular capillaries or arterioles and without typical biological signs. Clinical, biological, and outcome characteristics were similar between the cases described as having MPGN and TMA-like presentation. After a thorough
review of histopathological descriptions for each case, MPGN lesions seems to be the consequences of chronic glomerular endothelial injury in persistent TMA. We suspect that VEGF and IL-6 play a key role in the physiopathology of the spectrum of renal involvement from TMA-like to MPGN observed in TAFRO syndrome. Conclusion: We present a Caucasian iMCD patient with TAFRO syndrome with renal insufficiency secondary to MPGN, which might be secondary to a chronic TMA-like disease. We suspect that there is a continuum between TMA and MPGN lesions in TAFRO syndrome favored by VEGF and IL-6.