血液疾患と輸血ジャーナル

概要

Successful Treatment of Immunosuppressant-resistant Thrombocytopenia with Eltrombopag in a Patient with TAFRO Syndrome(Retracted)

Kiyoshi Okazuka, Nobuhiro Tsukada, Yoshitaka Isotani, Kota Sato, Kanji Miyazaki

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.