Comparison of the clinical characteristics of TAFRO syndrome and idiopathic multicentric Castleman disease in general internal medicine: a 6-year retrospective study.

Published on Feb 1, 2020in Internal Medicine Journal1.677
· DOI :10.1111/IMJ.14404
Yoshito Nishimura5
Estimated H-index: 5
(Okayama University),
Yoshihisa Hanayama6
Estimated H-index: 6
(Okayama University)
+ 2 AuthorsFumio Otsuka3
Estimated H-index: 3
(Okayama University)
BACKGROUND: Although thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome was first described as a variant of idiopathic multicentric Castleman disease, patients with TAFRO syndrome have more aggressive clinical features. Because these patients may present with fever of unknown origin, general physicians need to recognize its characteristic laboratory data and clinical features during hospitalization. AIMS: Herein, we described the features, symptoms, and characteristics of TAFRO syndrome and compared them to those of idiopathic Castleman disease. METHODS: This was a retrospective study of patients with histopathologically confirmed TAFRO syndrome and idiopathic multicentric Castleman disease who were diagnosed and managed between April 2012 and June 2018 in a Japanese university hospital General Medicine Department. RESULTS: We found that the hospitalizations were significantly longer among patients with TAFRO syndrome compared to those with idiopathic Castleman disease (median: 87 days; range: 34-236 days vs. median: 30 days; range: 13-59 days; p < 0.01). Patients with TAFRO syndrome were more likely to present with fever, abdominal pain, and elevated inflammatory markers and be misdiagnosed with an infectious disease during the first hospital visit. Approximately 40% of patients with TAFRO syndrome had no radiographically enlarged lymph nodes. CONCLUSIONS: TAFRO syndrome may present like an infectious disease with an aggressive clinical course. Our study highlights the importance of placing significance on chief complaints and laboratory data. Physicians need to recognize the clinical and laboratory features of this disease to avoid missing this potentially fatal disorder. This article is protected by copyright. All rights reserved.
📖 Papers frequently viewed together
4 Citations
58 Citations
#1Alvaro I. OrtizH-Index: 5
#2Pedro CárdenasH-Index: 1
Last. Jacobo OrtizH-Index: 2
view all 6 authors...
Background To report the neuro-ophthalmological findings in the TAFRO syndrome in a South American patient.
5 CitationsSource
#1Kazuya SakaiH-Index: 6
#2Takeshi MaedaH-Index: 1
Last. Yasunori UedaH-Index: 22
view all 6 authors...
AbstractThrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome is considered as a unique clinicopathologic variant of multicentric Castleman’s disease and is recently reported in Japan. This entity represents a severe inflammatory state leading to organ failures such as severe liver dysfunction seen in our case, and can be treated by immunosuppressive agents, steroids, and cyclosporine shown in several case reports. A systematic review and our case suggest the p...
24 CitationsSource
#1Aleksandar R. Zivkovic (University Hospital Heidelberg)H-Index: 11
#2Sebastian O. Decker (University Hospital Heidelberg)H-Index: 4
Last. Thorsten Brenner (University Hospital Heidelberg)H-Index: 15
view all 8 authors...
Early sepsis identification is of paramount importance for an effective therapy and the patient outcome; however, a suitable prognostic biomarker is lacking. Anti-inflammatory nonneuronal cholinergic signaling modulates the magnitude of an immune response. Serum cholinesterase (BChE), an enzyme that hydrolyzes acetylcholine, plays an important role during inflammatory response and serves as an accurate index of cholinergic activity. BChE activity was measured in septic patients using a point-of-...
12 CitationsSource
#1Fabrice CoutierH-Index: 1
Last. Nadine Magy-BertrandH-Index: 13
view all 8 authors...
TAFRO syndrome was first described as a variant of multicentric Castleman’s disease with thrombocytopenia, anasarca, fever, renal dysfunction, and organomegaly. We report the case of a 25-year-old Caucasian male with diagnosis of TAFRO syndrome and present a literature review. The objective of the study was to compare TAFRO syndrome between Japanese and non-Japanese patients. Cases were included by searching the term “TAFRO” in the Medline database using PubMed between 2010 and 2016. The Student...
7 CitationsSource
#1Weerapat Owattanapanich (MU: Mahidol University)H-Index: 6
#2Wikanda Pholmoo (MU: Mahidol University)H-Index: 1
Last. Noppadol Siritanaratkul (MU: Mahidol University)H-Index: 15
view all 4 authors...
Castleman’s disease (CD) is a rare lymphoproliferative disorder, and its prevalence in Thailand is not known. This 10-year period study investigated the prevalence of CD in Thailand, and the clinical characteristics and outcomes of Thai CD patients, with special focus on the existence and prevalence of TAFRO syndrome. TAFRO syndrome is defined as CD with thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. Thirty-three CD patients diagnosed and treated at Siriraj Hospital dur...
4 CitationsSource
#1Kentaro Sakashita (Tokyo Metropolitan Matsuzawa Hospital)H-Index: 5
#1Kentaro Sakashita (Tokyo Metropolitan Matsuzawa Hospital)H-Index: 2
Last. Mikio TakamoriH-Index: 2
view all 3 authors...
: Multicentric Castleman's disease (MCD), a distinct subtype of Castleman's disease, is a rare, nonneoplastic, lymphoproliferative disorder. Patients with MCD present with systemic symptoms and multiple lymphadenopathy. Lymph node biopsy is necessary for the diagnosis of various histological MCD patterns including hyaline vascular, plasma cell, and mixed types. Human herpesvirus 8 (HHV8) infection was identified as an important etiology of MCD among immunocompromised patients such as those posit...
10 CitationsSource
#1T. KiguchiH-Index: 1
#2C. SatoH-Index: 1
Last. Mitsuru Matsuki (Kindai University)H-Index: 22
view all 6 authors...
Aim To assess detailed computed tomography (CT) findings in patients with the recently described thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome, in order to contribute to imaging interpretation in the challenging diagnosis of this disease. Materials and methods The institutional review board approved this retrospective study and waived the need for informed consent. Eleven patients (six men, five women; mean age, 52.5 years) with confirmed TAFRO syndrome...
4 CitationsSource
#1Céline Louis (University of Geneva)H-Index: 1
#2Sandrine Elisabeth Vijgen (University of Geneva)H-Index: 6
Last. Yannick D. Muller (University of Geneva)H-Index: 18
view all 9 authors...
Background TAFRO syndrome has been reported in Japan among HHV-8-negative/idiopathic multicentric Castleman’s disease (iMCD) patients. To date, the majority of iMCD patients with TAFRO syndrome originate from Japan. Case presentation Herein, we report a 67-year-old HIV/HHV-8 negative Caucasian iMCD patient diagnosed with TAFRO. He presented with marked systemic inflammation, bicytopenia, terminal renal insufficiency, diffuse lymphadenopathies and anasarca. Lymph node and bone marrow biopsies rev...
14 CitationsSource
#1David C. Fajgenbaum (UPenn: University of Pennsylvania)H-Index: 16
#2Thomas S. Uldrick (NIH: National Institutes of Health)H-Index: 30
Last. Megan S. Lim (UPenn: University of Pennsylvania)H-Index: 55
view all 38 authors...
Human herpesvirus-8 (HHV-8)–negative, idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening disorder involving systemic inflammatory symptoms, polyclonal lymphoproliferation, cytopenias, and multiple organ system dysfunction caused by a cytokine storm often including interleukin-6. iMCD accounts for one third to one half of all cases of MCD and can occur in individuals of any age. Accurate diagnosis is challenging, because no standard diagnostic criteria or diagnostic b...
147 CitationsSource
#1Fabio Freire JoséH-Index: 2
Last. Nelson HamerschlakH-Index: 26
view all 10 authors...
AbstractRationale:This is the report of the first case of TAFRO syndrome (Thrombocytopenia, Anasarca, myelofibrosis, Renal dysfunction, Organomegaly) in Latin America.Patient concerns:The patient was a 61-year-old white woman of Ashkenazi Jewish descent, who presented with a history of 8 days of nau
24 CitationsSource
Cited By5
#1Kosuke Oka (Okayama University)H-Index: 2
#2Mai Yamane (Okayama University)
Last. Fumio Otsuka (Okayama University)H-Index: 35
view all 9 authors...
Abstract TAFRO syndrome is a rare variant of idiopathic multicentric Castleman's disease, for which disseminated non-tuberculous mycobacteria (NTM) infection must be excluded. However, due to the slow and fastidious growth of the organisms, identification of the pathogen is often challenging. We herein describe a case of disseminated Mycobacterium genavence infection, in which manifestations of the patient were confusingly similar to those of TAFRO syndrome. A 69-year-old Japanese man presented ...
#1Peng Xia (Peking Union Medical College Hospital)H-Index: 2
#2Lu Zhang (Peking Union Medical College Hospital)H-Index: 1
Last. Limeng Chen (Peking Union Medical College Hospital)H-Index: 10
view all 11 authors...
INTRODUCTION Thrombocytopenia, ascites, myelofibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome is a newly recognized and rare clinical subtype of Castleman disease. Renal involvement in TAFRO syndrome usually presents with mild proteinuria, microscopic hematuria, and acute renal injury requiring temporary renal replacement. There is no standard therapy available and treatment failures are common, leading to a poor prognosis. We report a case of acute renal failure caused by TAFRO sy...
1 CitationsSource
#1Sylvain Raoul Simeni Njonnou (University of Dschang)H-Index: 1
Last. Virginie De WildeH-Index: 4
view all 5 authors...
TAFRO (thrombocytopenia, anasarca, fever, reticulin myelofibrosis or renal dysfunction and organomegaly) syndrome is a systemic inflammatory disease characterised by thrombocytopenia, anasarca, fever or inflammatory syndrome, reticulin myelofibrosis or renal dysfunction and organomegaly. It was first described as a subtype of idiopathic multicentric Castleman disease. Here, we report the case of a 42-year-old woman presenting with thrombocytopenia, anasarca, inflammatory syndrome, renal insuffic...
2 CitationsSource
#1Angela Dispenzieri (Mayo Clinic)H-Index: 138
#2David C. Fajgenbaum (UPenn: University of Pennsylvania)H-Index: 16
Castleman disease (CD) describes a group of at least four disorders that share a spectrum of characteristic histopathological features but have a wide range of etiologies, presentations, treatments, and outcomes. CD includes unicentric CD (UCD) and multicentric CD (MCD), the latter of which is divided into idiopathic MCD (iMCD), HHV8-associated MCD (HHV8-MCD), and POEMS-associated MCD. iMCD can be further sub-classified into iMCD-TAFRO (thrombocytopenia, ascites, reticulin fibrosis, renal dysfun...
20 CitationsSource
#1Yi Zhang (ZJU: Zhejiang University)H-Index: 2
#2Shanshan Suo (ZJU: Zhejiang University)H-Index: 6
Last. Jie Jin (ZJU: Zhejiang University)H-Index: 59
view all 8 authors...
Castleman disease (CD) is a rare polyclonal lymphoproliferative disorder with unknown etiology. TAFRO syndrome is now regarded as a specific subtype of CD, and is still a huge challenge for clinicians. To clarify the clinical features and management of TAFRO syndrome in China, we retrospectively analyzed 96 patients with HIV-negative CD (52 with unicentric CD and 44 with multicentric CD), who were diagnosed and treated at our center between 2008 and 2017. Specially, we systematically reviewed th...
2 CitationsSource