High proportion of TAFRO syndrome in Thai adult Castleman’s disease patients: a 10-year experience
| Autor: | Noppadol Siritanaratkul, Wikanda Pholmoo, Weerapat Owattanapanich, Tawatchai Pongpruttipan |
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| Rok vydání: | 2018 |
| Předmět: |
Adult
Male medicine.medical_specialty Fever Pleural effusion Treatment outcome Castleman’s disease Lymphadenopathy Disease Severity of Illness Index Anasarca Gastroenterology Organomegaly 03 medical and health sciences 0302 clinical medicine Internal medicine Antineoplastic Combined Chemotherapy Protocols Ascites Prevalence medicine Edema Humans Hospitals Teaching Hematology business.industry Castleman Disease Remission Induction TAFRO syndrome General Medicine Middle Aged Prognosis Thailand medicine.disease Survival Analysis Thrombocytopenia Reticulin fibrosis 030220 oncology & carcinogenesis Female Lost to Follow-Up Original Article medicine.symptom business Follow-Up Studies 030215 immunology |
| Zdroj: | Annals of Hematology |
| ISSN: | 1432-0584 0939-5555 |
| DOI: | 10.1007/s00277-018-3269-x |
| Popis: | 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 poor outcome. |
| Databáze: | OpenAIRE |
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