Eltrombopag成功治疗TAFRO综合征患者的免疫抑制剂抵抗性血小板减少症(缩回)

K. Okazuka, N. Tsukada, Yoshitaka Isotani, Kota Sato, Kanji Miyazaki, Y. Abe, Y. Yoshiki, T. Ishida, Kenshi Suzuki
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引用次数: 0

摘要

TAFRO综合征被认为是一种引起血小板减少的全身性炎症性疾病;全身水肿;发烧;肾功能不全;器官肿大,包括肝脾肿大和淋巴结肿大。它可能是多中心Castleman病(MCD)的一种变体。虽然TARFO综合征的淋巴结病理表现与MCD相似,但TAFRO综合征的临床表现与MCD不同;即,TAFRO综合征会导致严重的血小板减少和全身水肿,但不会导致多克隆性γ病,也不会导致HHV-8阳性。TAFRO综合征的病因尚未阐明。在我们的病例中,一些症状通过托珠单抗得到了解决,但患者的严重血小板减少症对各种治疗都有耐药性。虽然TAFRO综合征中血小板减少的发病机制尚不清楚,但在我们的病例中,血小板生成素受体激动剂eltrombopag改善了tocilizumab耐药的血小板减少。对于TAFRO综合征持续性血小板减少症患者,伊曲波帕可能是一种有效的治疗方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Successful Treatment of Immunosuppressant-resistant Thrombocytopenia with Eltrombopag in a Patient with TAFRO Syndrome(Retracted)
TAFRO syndrome has been proposed to be a systemic inflammatory disorder that causes thrombocytopenia; anasarca; fever; renal insufficiency; and organomegaly, including hepatosplenomegaly and lymphadenopathy. It could be a variant of multicentric Castleman disease (MCD). Although the pathological findings of the lymph nodes in TARFO syndrome resemble those seen in MCD, the clinical findings of TAFRO syndrome differ from those of MCD; i.e., TAFRO syndrome causes severe thrombocytopenia and systemic edema, but not polyclonal gammopathy and no positivity of HHV-8. The etiology of TAFRO syndrome has not been elucidated. In our case, several symptoms were resolved with tocilizumab, but the patient’s severe thrombocytopenia was resistant to various treatments. Although the pathogenesis of thrombocytopenia in TAFRO syndrome remains unclear, in our case eltrombopag, which is a thrombopoietin receptor agonist, improved tocilizumab-resistant thrombocytopenia. Eltrombopag could be an effective treatment for persistent thrombocytopenia in TAFRO syndrome.
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