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

ABSTRACT

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.