摘要
To the Editor: Castleman's disease (CD) describes a group of lymphoproliferative disorders with heterogeneous clinical manifestations.[1] CD comprises at least two distinct diseases (unicentric CD [UCD] and multicentric CD [MCD]) with respective clinical features and prognoses: UCD often involves one lymph node area with favorable outcome: MCD is a systemic disease with relatively poor prognosis which involves multiple lymph node areas with constitutional symptoms (e.g. fever, night sweats, and weakness) and organ dysfunction. According to pathogenesis, MCD can be further divided into different subtypes based on the status of human herpes virus-8 (HHV-8) infection. Of the HHV-8 negative MCD (also named as idiopathic MCD [iMCD]), there is a peculiar subtype named TAFRO syndrome which constitutes a constellation of unique clinical manifestations (T: Thrombocytopenia, A: Anasarca, F: myelofibrosis, R: Renal dysfunction, and O: Organomegaly). This syndrome, first reported in Japan[2] has been reported mostly in the Japanese population and occasionally in Caucasians. There is no such case reported in China. We herein report the first patient with TAFRO syndrome in China with confirmed myelofibrosis.
To the Editor: Castleman's disease (CD) describes a group of lymphoproliferative disorders with heterogeneous clinical manifestations.[1] CD comprises at least two distinct diseases (unicentric CD [UCD] and multicentric CD [MCD]) with respective clinical features and prognoses: UCD often involves one lymph node area with favorable outcome: MCD is a systemic disease with relatively poor prognosis which involves multiple lymph node areas with constitutional symptoms (e.g. fever, night sweats, and weakness) and organ dysfunction. According to pathogenesis, MCD can be further divided into different subtypes based on the status of human herpes virus-8 (HHV-8) infection. Of the HHV-8 negative MCD (also named as idiopathic MCD [iMCD]), there is a peculiar subtype named TAFRO syndrome which constitutes a constellation of unique clinical manifestations (T: Thrombocytopenia, A: Anasarca, F: myelofibrosis, R: Renal dysfunction, and O: Organomegaly). This syndrome, first reported in Japan[2] has been reported mostly in the Japanese population and occasionally in Caucasians. There is no such case reported in China. We herein report the first patient with TAFRO syndrome in China with confirmed myelofibrosis.
作者
Wan-Lu Ma
Lu Zhang
Tie-Nan Zhu
Dao-Bin Zhou
Jian Liz
Jian Sun
Bo-Ju Pan
Wei-Xing Xu
Wan-Lu Ma;Lu Zhang;Tie-Nan Zhu;Dao-Bin Zhou;Jian Li;Jian Sun;Bo-Ju Pan;Wei-Xing Xu(Department of Endocrinology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China;Department of Hematology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China;Department of Pathology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China 4Department of Hematology,Cangzhou Central Hospital,Cangzhou,Hebei 061001,China)