Furuto Yoshitaka, Hashimoto Hirotsugu, Horiuti Hajime, Shibuya Yuko
Department of Hypertension and Nephrology Department of Diagnostic Pathology, NTT Medical Centre Tokyo, Higasi-Gotanda, Shinagawa-ku, Tokyo, Japan.
Medicine (Baltimore). 2018 Jun;97(24):e11057. doi: 10.1097/MD.0000000000011057.
TAFRO syndrome is a systemic inflammatory disease proposed recently from Japan. The cause of TAFRO syndrome is unclear. Moreover, the disease characteristics and kidney pathology are yet unknown well and there are few cases. Herein, we report a patient with TAFRO syndrome and present the features of the renal histopathology.
A 55-year-old woman presented to our hospital with the main complaint of subacute dyspnoea.
Physical findings included a low-grade fever and generalised oedema. A blood test showed anaemia, coagulation abnormalities, hypoproteinaemia, impaired renal function, proteinuria, and elevated alkaline phosphatase (ALP), C-reactive protein (CRP), interleukin-6 (IL-6). Chest and abdominal computed tomography showed an anterior mediastinal mass and multiple enlarged lymph nodes.
Nephrotic syndrome secondary to a malignant mediastinal tumour was suspected; therefore, the patient underwent resection of the anterior mediastinal mass. Histopathological examination of the resected specimen showed lymphocytic proliferation without signs of malignancy. These findings were compatible with hyaline vascular type Castleman disease (CD), and with the associated multiple lymph nodes enlargement, the patient was initially diagnosed with multicenteric CD.
After resection of the whole tumour, all the clinical symptoms improved. However, after resection 6 months passed, the patient developed thrombocytopenia, anaemia, renal dysfunction, further enlargement of the residual lymph nodes, hepatosplenomegaly, and mild myelofibrosis. A diagnosis of TAFRO syndrome (TS) was eventually made. All symptoms improved with initial intravenous pulse steroid therapy followed by oral steroids. Histopathological examination of the renal biopsy samples showed findings resembling membranoproliferative glomerulonephritis (MPGN).
In TS, all characteristic signs may not exist from the beginning. The association between TS and CD is not clear. When we compared our findings with previously published cases of TS and CD, we found that the renal pathology findings resembled MPGN in many cases of TS, while only a few cases showed amyloidosis. Recent results suggest that TS may be an independent disease from CD, and given the frequency of renal pathology findings, it may also have a different aetiology. To the best of our knowledge, this case report is rare to demonstrate the renal pathology in a patient with conventional TAFRO syndrome.
TAFRO综合征是一种最近在日本提出的全身性炎症性疾病。TAFRO综合征的病因尚不清楚。此外，该疾病的特征和肾脏病理学情况仍鲜为人知，且病例较少。在此，我们报告一例TAFRO综合征患者，并呈现其肾脏组织病理学特征。
一名55岁女性因亚急性呼吸困难为主诉前来我院就诊。
体格检查发现低热和全身性水肿。血液检查显示贫血、凝血异常、低蛋白血症、肾功能受损、蛋白尿以及碱性磷酸酶（ALP）、C反应蛋白（CRP）、白细胞介素-6（IL-6）升高。胸部和腹部计算机断层扫描显示前纵隔肿块和多个肿大淋巴结。
怀疑为恶性纵隔肿瘤继发的肾病综合征；因此，患者接受了前纵隔肿块切除术。切除标本的组织病理学检查显示淋巴细胞增生，无恶性迹象。这些发现符合透明血管型Castleman病（CD），且伴有多个淋巴结肿大，患者最初被诊断为多中心性CD。
切除整个肿瘤后，所有临床症状均有改善。然而，切除6个月后，患者出现血小板减少、贫血、肾功能不全、残留淋巴结进一步肿大、肝脾肿大以及轻度骨髓纤维化。最终诊断为TAFRO综合征（TS）。最初通过静脉脉冲类固醇治疗，随后口服类固醇，所有症状均有改善。肾脏活检样本的组织病理学检查显示出类似膜增生性肾小球肾炎（MPGN）的表现。
在TS中，并非一开始就会出现所有特征性体征。TS与CD之间的关联尚不清楚。当我们将我们的发现与先前发表的TS和CD病例进行比较时，我们发现TS的许多病例中肾脏病理学发现类似于MPGN，而只有少数病例显示淀粉样变性。最近的结果表明，TS可能是一种独立于CD的疾病，鉴于肾脏病理学发现的频率，它可能也有不同的病因。据我们所知，本病例报告罕见地展示了传统TAFRO综合征患者的肾脏病理学情况。
