Cargando…

An anterior mediastinal lesion in TAFRO syndrome showing complete remission after glucocorticoid and tocilizumab therapy

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Sakashita, Kentaro, Murata, Kengo, Inagaki, Yuji, Oota, Souichi, Takamori, Mikio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons, Ltd 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4970309/
https://www.ncbi.nlm.nih.gov/pubmed/27516889
http://dx.doi.org/10.1002/rcr2.173
_version_ 1782445955933536256
author Sakashita, Kentaro
Murata, Kengo
Inagaki, Yuji
Oota, Souichi
Takamori, Mikio
author_facet Sakashita, Kentaro
Murata, Kengo
Inagaki, Yuji
Oota, Souichi
Takamori, Mikio
author_sort Sakashita, Kentaro
collection PubMed
description 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.
format Online
Article
Text
id pubmed-4970309
institution National Center for Biotechnology Information
language English
publishDate 2016
publisher John Wiley & Sons, Ltd
record_format MEDLINE/PubMed
spelling pubmed-49703092016-08-11 An anterior mediastinal lesion in TAFRO syndrome showing complete remission after glucocorticoid and tocilizumab therapy Sakashita, Kentaro Murata, Kengo Inagaki, Yuji Oota, Souichi Takamori, Mikio Respirol Case Rep Case Reports 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. John Wiley & Sons, Ltd 2016-07-04 /pmc/articles/PMC4970309/ /pubmed/27516889 http://dx.doi.org/10.1002/rcr2.173 Text en © 2016 The Authors. Respirology Case Reports published by John Wiley & Sons Australia, Ltd on behalf of The Asian Pacific Society of Respirology This is an open access article under the terms of the Creative Commons Attribution‐NonCommercial (http://creativecommons.org/licenses/by-nc/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.
spellingShingle Case Reports
Sakashita, Kentaro
Murata, Kengo
Inagaki, Yuji
Oota, Souichi
Takamori, Mikio
An anterior mediastinal lesion in TAFRO syndrome showing complete remission after glucocorticoid and tocilizumab therapy
title An anterior mediastinal lesion in TAFRO syndrome showing complete remission after glucocorticoid and tocilizumab therapy
title_full An anterior mediastinal lesion in TAFRO syndrome showing complete remission after glucocorticoid and tocilizumab therapy
title_fullStr An anterior mediastinal lesion in TAFRO syndrome showing complete remission after glucocorticoid and tocilizumab therapy
title_full_unstemmed An anterior mediastinal lesion in TAFRO syndrome showing complete remission after glucocorticoid and tocilizumab therapy
title_short An anterior mediastinal lesion in TAFRO syndrome showing complete remission after glucocorticoid and tocilizumab therapy
title_sort anterior mediastinal lesion in tafro syndrome showing complete remission after glucocorticoid and tocilizumab therapy
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4970309/
https://www.ncbi.nlm.nih.gov/pubmed/27516889
http://dx.doi.org/10.1002/rcr2.173
work_keys_str_mv AT sakashitakentaro ananteriormediastinallesionintafrosyndromeshowingcompleteremissionafterglucocorticoidandtocilizumabtherapy
AT muratakengo ananteriormediastinallesionintafrosyndromeshowingcompleteremissionafterglucocorticoidandtocilizumabtherapy
AT inagakiyuji ananteriormediastinallesionintafrosyndromeshowingcompleteremissionafterglucocorticoidandtocilizumabtherapy
AT ootasouichi ananteriormediastinallesionintafrosyndromeshowingcompleteremissionafterglucocorticoidandtocilizumabtherapy
AT takamorimikio ananteriormediastinallesionintafrosyndromeshowingcompleteremissionafterglucocorticoidandtocilizumabtherapy
AT sakashitakentaro anteriormediastinallesionintafrosyndromeshowingcompleteremissionafterglucocorticoidandtocilizumabtherapy
AT muratakengo anteriormediastinallesionintafrosyndromeshowingcompleteremissionafterglucocorticoidandtocilizumabtherapy
AT inagakiyuji anteriormediastinallesionintafrosyndromeshowingcompleteremissionafterglucocorticoidandtocilizumabtherapy
AT ootasouichi anteriormediastinallesionintafrosyndromeshowingcompleteremissionafterglucocorticoidandtocilizumabtherapy
AT takamorimikio anteriormediastinallesionintafrosyndromeshowingcompleteremissionafterglucocorticoidandtocilizumabtherapy