title: An anterior mediastinal lesion in TAFRO syndrome showing complete remission after glucocorticoid and tocilizumab therapy.
abstract: Thrombocytopenia (T), anasarca (A), myelofibrosis (F), renal dysfunction (R), and organomegaly (O) (TAFRO) syndrome is a variant of multicentric Castleman's disease. We describe here a 57-year-old man who presented with persistent fever, pleural effusion, and ascites. He was negative for human immunodeficiency virus and human herpes virus-8. A computed tomography scan showed an anterior mediastinal mass and small inguinal lymphadenopathy. Although a biopsy of the anterior mediastinum showed fatty tissue infiltrated with CD20 (+) and CD45RO (+) lymphocytes, a biopsy of the left inguinal lymph node revealed a hyaline vascular type of Castleman's disease. He subsequently developed severe thrombocytopenia and renal dysfunction. In addition, his bone marrow biopsy showed myelofibrosis. TAFRO syndrome was diagnosed based on the lymph node pathology and the characteristic manifestations of the syndrome. Tocilizumab and glucocorticoid therapy achieved complete remission and regression of the mediastinal mass. To our knowledge, this is the first report of TAFRO syndrome accompanied by an anterior mediastinal mass, which responded very well to therapy.
authors: Department of Respiratory Medicine Tokyo Metropolitan Tama Medical Center Fuchu-shi Tokyo Japan.
authors: Kentaro
authors: 0000-0002-3948-5557
authors: K
authors: Sakashita
authors: Department of Respiratory Medicine Tokyo Metropolitan Tama Medical Center Fuchu-shi Tokyo Japan.
authors: Kengo
authors: K
authors: Murata
authors: Department of Respiratory Medicine Tokyo Metropolitan Tama Medical Center Fuchu-shi Tokyo Japan.
authors: Yuji
authors: Y
authors: Inagaki
authors: Department of Rheumatology and Connective Tissue Disease Tokyo Metropolitan Bokutoh Hospital Sumida-ku Tokyo Japan.
authors: Souichi
authors: S
authors: Oota
authors: Department of Respiratory Medicine Tokyo Metropolitan Tama Medical Center Fuchu-shi Tokyo Japan.
authors: Mikio
authors: M
authors: Takamori
keywords: Anterior mediastinal mass; TAFRO syndrome; multicentric Castleman's disease; tocilizumab