Clinicopathologic analysis of TAFRO syndrome demonstrates a distinct subtype of HHV-8-negative multicentric Castleman disease

American Journal of Hematology
Noriko IwakiYasuharu Sato

Abstract

Multicentric Castleman disease (MCD) describes a heterogeneous group of disorders involving systemic inflammation, characteristic lymph node histopathology, and multi-organ dysfunction because of pathologic hypercytokinemia. Whereas Human Herpes Virus-8 (HHV-8) drives the hypercytokinemia in a cohort of immunocompromised patients, the etiology of HHV-8-negative MCD is idiopathic (iMCD). Recently, a limited series of iMCD cases in Japan sharing a constellation of clinical features, including thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O) has been described as TAFRO syndrome. Herein, we report clinicopathological findings on 25 patients (14 males and 11 females; 23 Japanese-born and two US-born), the largest TAFRO syndrome case series, including the first report of cases from the USA. The median age of onset was 50 years old (range: 23-72). The frequency of each feature was as follows: thrombocytopenia (21/25), anasarca (24/25), fever (21/25), organomegaly (25/25), and reticulin fibrosis (13/16). These patients frequently demonstrated abdominal pain, elevated serum alkaline phosphatase levels, and acute kidney failure. Surprisingly, none of the cases demonstrated marked hypergammoglobu...Continue Reading

References

Aug 1, 1989·Proceedings of the National Academy of Sciences of the United States of America·T IshibashiS A Burstein
Sep 1, 1985·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·G FrizzeraA Goldman
Nov 1, 1982·Arthritis and Rheumatism·E M TanR J Winchester
Jul 1, 1956·Cancer·B CASTLEMANV P MENENDEZ
Mar 15, 2005·Acta Oncologica·Ashita Waterston, Mark Bower
Feb 28, 2007·The American Journal of Surgical Pathology·Andreas KreftCharles James Kirkpatrick
May 24, 2008·International Journal of Surgical Pathology·Masaru KojimaShigeo Nakamura
Jun 30, 2010·Clinical Infectious Diseases : an Official Publication of the Infectious Diseases Society of America·Thomas S UldrickRobert Yarchoan
Mar 1, 2012·Annals of Surgery·Nadia TalatKlaus-Martin Schulte
May 2, 2012·Blood·Angela Dispenzieri
Apr 19, 2013·American Journal of Clinical Pathology·Girish VenkataramanIrina Maric
Jun 27, 2013·Journal of Clinical and Experimental Hematopathology : JCEH·Kazue TakaiTakako Saeki
Jun 27, 2013·Journal of Clinical and Experimental Hematopathology : JCEH·Noriko IwakiTadashi Yoshino
Feb 18, 2014·American Journal of Hematology·Angela Dispenzieri

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Citations

Apr 12, 2016·The Lancet. Haematology·Amy Y LiuDavid C Fajgenbaum
Jun 15, 2016·Seminars in Diagnostic Pathology·Hao-Wei WangElaine S Jaffe
Jun 2, 2016·International Journal of Hematology·Shunichiro YasudaTetsuya Fukuda
Nov 18, 2016·Annals of Hematology·Fatemeh BehniaDavid C Fajgenbaum
Apr 21, 2018·American Journal of Hematology·Sheila K PiersonDavid C Fajgenbaum
Oct 5, 2018·Modern Rheumatology·Tomohiro KogaKazuyuki Yoshizaki
Jul 19, 2017·Case Reports in Critical Care·Guillaume MorelKhaldoun Kuteifan
Jun 22, 2019·American Journal of Hematology·Shino FujimotoYasufumi Masaki
Jun 23, 2020·Pediatric Blood & Cancer·Shogo MinamikawaRyojiro Tanaka
Sep 8, 2020·American Journal of Hematology·David C FajgenbaumUNKNOWN Castleman Disease Collaborative Network Scientific Advisory Board diagnostic criteria international working group and treatm
Nov 17, 2017·British Journal of Haematology·Eric OksenhendlerLionel Galicier
Nov 11, 2017·Acute Medicine & Surgery·Masatoshi OkumuraHideki Shimaoka
Feb 14, 2020·Oncogene·Akihide YoshimiWenbin Xiao
Mar 1, 2020·American Journal of Clinical Pathology·Karen M Chisholm, Mark D Fleming
Feb 28, 2020·Blood·Angela Dispenzieri, David C Fajgenbaum
Sep 24, 2020·Annals of Hematology·Mitsuhiro AkiyamaTsutomu Takeuchi
Sep 24, 2018·CEN Case Reports·Keiko HashimotoNorihiro Nakata
Nov 30, 2019·International Journal of Hematology·Yasufumi MasakiUNKNOWN Japanese TAFRO Syndrome Research Team
Jan 2, 2020·Clinical Case Reports·Zi Ying LiRaza Mian

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Castleman Disease

Castleman disease is a rare disorder that involves an overgrowth of cells in the lymph nodes. Unicentric Castleman disease affects one lymph node, usually in the chest or abdomen. Multicentric Castleman disease affects multiple lymph nodes, commonly located in the neck, collarbone, underarm and groin areas. Discover the latest research on Castleman disease here.