A comparison of TAFRO syndrome between Japanese and non-Japanese cases: a case report and literature review

• Published in: Annals of hematology Vol. 97; no. 3; pp. 401 - 407
• Main Authors: Coutier, Fabrice, Meaux Ruault, Nadine, Crepin, Thomas, Bouiller, Kevin, Gil, Helder, Humbert, Sébastien, Bedgedjian, Isabelle, Magy-Bertrand, Nadine
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.03.2018; Springer Nature B.V
• Subjects: Adult; Case reports; Castleman Disease - complications; Castleman Disease - ethnology; Castleman Disease - pathology; Cornea; Edema - complications; Edema - ethnology; Edema - pathology; Fever - complications; Fever - ethnology; Fever - pathology; Hands; Hematology; Humans; Hypertrophy - complications; Hypertrophy - pathology; Japan; Kidney Diseases - complications; Kidney Diseases - ethnology; Kidney Diseases - pathology; Literature reviews; Male; Medicine; Medicine & Public Health; Oncology; Review Article; Syndrome; Thrombocytopenia - complications; Thrombocytopenia - ethnology; Thrombocytopenia - pathology; TAFRO; Castleman; Ethnic differences; Japanese
• ISSN: 0939-5555; 1432-0584
• DOI: 10.1007/s00277-017-3138-z

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 t test and Mann-Whitney U test were used to compare continuous variables. Fisher’s exact test was used for categorical variables. Statistical significance was set at p  < 0.05. Forty-four cases were included. Thirty-two patients (73%) were of Japanese origin. Japanese patients were significantly older than non-Japanese ones (52.0 ± 13.6 years versus 36.9 ± 19.8 years, p  = 0.0064) but there was no difference in gender. Creatinine level on admission was significantly higher in the non-Japanese group (1.87 ± 0.84 mg/dL versus 1.32 ± 0.57 mg/dL, p  = 0.0347). There were no significant differences concerning lymphadenopathy, elevated number of megakaryocytes on bone marrow aspiration, autoimmune abnormalities, and the following parameters on admission: platelet count, hemoglobin, albumin, alkaline phosphatase (ALP). Corticotherapy was always used on induction for Japanese patients while it was only used in 75% of the cases on induction in non-Japanese patients ( p  = 0.0166). Our study was the first to compare TAFRO syndrome according to ethnicity. Japanese patients were significantly older and had a significantly lower creatinine level on admission than non-Japanese patients.