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A Case of TAFRO Syndrome Maintained in Remission for 5 Years After Discontinuation of Tocilizumab.

TAFRO syndrome is a subtype of idiopathic multicentric Castleman's disease (iMCD) that is characterized by thrombocytopenia, generalized edema, fever, bone marrow fibrosis, renal failure, and organ enlargement, and has a poor prognosis. The prognosis of TAFRO syndrome is worse than that of iMCD-not otherwise specified (iMCD-NOS), with a high mortality rate. There are only few long term follow-up reports after remission of TAFRO syndrome with tocilizumab (TCZ) treatment in a patient in whom all drugs were discontinued after attaining sustained remission. Here, we report a case in which interleukin (IL) -6 negativity was confirmed and remission was maintained without relapse for 5 years after all drug treatment, including TCZ, was discontinued.