Fatal case of TAFRO syndrome associated with over-immunosuppression: a case report and review of the literature.

Fatal case of TAFRO syndrome associated with over-immunosuppression: a case report and review of the literature. Nagoya J Med Sci. 2019 Aug;81(3):519-528 Authors: Matsuhisa T, Takahashi N, Nakaguro M, Sato M, Inoue E, Teshigawara S, Ozawa Y, Kondo T, Nakamura S, Sato J, Ban N Abstract TAFRO syndrome is a novel disease concept characterized by Thrombocytopenia, Anasarca, myeloFibrosis, Renal dysfunction, Organomegaly, multiple lymphadenopathy and a histopathological pattern of atypical Castleman's disease. A 58-year-old man was diagnosed as TAFRO syndrome by clinical and histopathological findings. After receiving intensive immunosuppressive therapy, his thrombocytopenia and anasarca had not improved. He developed complications such as methicillin-resistant Staphylococcus aureus sepsis, gastrointestinal bleeding, peritonitis caused by Stenotrophomonas maltophilia, gastrointestinal perforation, and disseminated candidiasis resulting in death. Autopsy revealed disseminated candidiasis and hemophagocytic lymphohistiocytosis, with no evidence of TAFRO syndrome. During treatment, we regarded his lasting thrombocytopenia and anasarca as insufficient control of TAFRO syndrome. However, the autopsy revealed that thrombocytopenia was caused by secondary hemophagocytic lymphohistiocytosis caused by over-immunosuppression. We reviewed the published literature to identify indicators of adequate treatment, which suggested improvement of platelet c...
Source: Nagoya Journal of Medical Science - Category: International Medicine & Public Health Tags: Nagoya J Med Sci Source Type: research