Successful treatment of TAFRO syndrome, a variant type of multicentric Castleman disease with thrombotic microangiopathy, with anti-IL-6 receptor antibody and steroids
Successful treatment of TAFRO syndrome, a variant type of multicentric Castleman disease with thrombotic microangiopathy, with anti-IL-6 receptor antibody and steroids
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DOI：
10.1007/s12185-016-1978-2
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发表时间：
2016-06-01
影响因子：
2.1
通讯作者：
Nosaka, Kisato
中科院分区：
文献类型：
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作者：
Fujiwara, Shiho;Mochinaga, Hiromi;Nosaka, Kisato
TAFRO综合征是一种罕见的多中心Castleman病的变异型，其特征是血小板减少、无鞭痕、骨髓网状细胞纤维化、肾功能障碍和器官肿大。在这里，我们报告了一例TAFRO综合征，成功地用tocilizumab治疗。本院收治一位50岁男性，以发热、上腹痛、腹胀及四肢大量浮肿为主诉。计算机断层扫描显示双侧胸腔积液、腹水和淋巴结病。实验室数据显示肾功能不全、贫血和血小板减少。骨髓和颈部淋巴结检查导致诊断为透明血管型Castleman病。血清IL-6水平极高。TAFRO综合征最终确诊。患者每周接受tocilizumab治疗，tocilizumab是一种抗IL-6受体抗体和类固醇。4周后症状消失，血清IL-6水平恢复正常。ADAMTS13(一种含有凝血酶反应蛋白1型基序的去整合素和金属蛋白酶，成员13)活性在治疗前显著降低(9.9%)，在tocilizumab治疗后升高。本病例提示tocilizumab是治疗TAFRO综合征的有效药物。我们认为TAFRO综合征的高细胞分裂素血症抑制了ADAMTS13的活性，从而导致血栓性微血管病变。
TAFRO syndrome is a rare variant type of multicentric Castleman disease, which is characterized by thrombocytopenia, anasarca, reticulin fibrosis of bone marrow, renal dysfunction and organomegaly. Here, we report a case of TAFRO syndrome that was successfully treated with tocilizumab. A 50-year-old man, who presented with fever, epigastric pain, abdominal fullness, and massive edema of the extremities, was admitted to our hospital. Computed tomography revealed bilateral pleural effusions, ascites, and lymphadenopathy. Laboratory data showed renal dysfunction, anemia, and thrombocytopenia. Examination of bone marrow and cervical lymph nodes led to a diagnosis of hyaline vascular-type Castleman disease. The level of serum interleukin (IL)-6 was extremely high. TAFRO syndrome was finally diagnosed. The patient was treated weekly with tocilizumab, an anti-IL-6 receptor antibody and steroids. In 4 weeks, all symptoms disappeared and serum IL-6 level returned to normal. Activity of ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), which was significantly decreased (9.9 %) prior to treatment, increased after treatment with tocilizumab. The present case suggests that tocilizumab is an effective therapeutic agent for TAFRO syndrome. We suggest that hypercytokinemia in TAFRO syndrome inhibits ADAMTS13 activity, thereby inducing thrombotic microangiopathy.