High proportion of TAFRO syndrome in Thai adult Castleman's disease patients: a 10-year experience

Annals of Hematology
Weerapat OwattanapanichNoppadol Siritanaratkul

Abstract

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 during January 2007 to December 2016 were included. The prevalence of CD was 1.4 per 1,000,000 patients/10 years. Median age was 46 years, with slight female predominance. Six patients were assigned to the TAFRO group. A high proportion of TAFRO syndrome (18.2%) was found among Thai adult CD patients. In addition to routine TAFRO diagnostic criteria, significantly lower hemoglobin and albumin levels were observed in the TAFRO group than in the non-TAFRO group. Treatment outcomes of CD patients were complete remission (52%), stable disease (30%), and death (13%). Three-year overall survival in the non-TAFRO group and TAFRO group was 88 and 50%, respectively. While most CD patients had a good prognosis, severe cases with TAFRO syndrome had po...Continue Reading

References

Jul 19, 2011·The Oncologist·Nadia Talat, Klaus-Martin Schulte
Mar 22, 2013·Pathology Oncology Research : POR·Györgyi MűzesLídia Sréter
Jun 27, 2013·Journal of Clinical and Experimental Hematopathology : JCEH·Noriko IwakiTadashi Yoshino
Nov 17, 2017·British Journal of Haematology·Eric OksenhendlerLionel Galicier

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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.