Multicentric Castleman's disease is a systemic inflammatory disorder 
characterized by lymphadenopathy and excessive interleukin-6 production. A 
unique clinicopathologic variant of multicentric Castleman's disease, TAFRO 
(i.e., thrombocytopenia, anasarca, fever, renal failure or reticulin fibrosis, 
and organomegaly) syndrome, was recently proposed in Japan. Despite the 
successful use of anti-interleukin-6 therapy in some patients with TAFRO 
syndrome, not all patients achieve remission. The pathophysiological etiology of 
and suitable therapeutic strategies for this variant have not been established. 
Here, we present our experience of a unique case of TAFRO syndrome in a 
78-year-old woman whose symptoms responded differently to several therapies. 
Tocilizumab, an anti-interleukin-6 receptor antibody, successfully induced 
remission of fever and lymphadenopathy. However, severe thrombocytopenia 
persisted and she developed anasarca, ascites, and pleural effusion shortly 
thereafter. Rituximab, an anti-CD20 antibody, and glucocorticoid therapy 
provided no symptom relief. In contrast, cyclosporine A, an immunosuppressive 
agent that blocks T cell function by inhibiting interleukin-2, yielded immediate 
improvements in systemic fluid retention and a gradual increase in platelet 
count, with complete resolution of disease symptoms. Excessive serum 
interleukin-2, when used as an anti-cancer agent, has been reported to cause 
side effects such as fluid retention, thrombocytopenia, and renal failure. Our 
case was unique because the anti-interleukin-2 therapy successfully improved 
symptoms that were not relieved with anti-interleukin-6 therapy. The present 
report therefore provides insight into the possible role of interleukin-2, in 
addition to interleukin-6, in TAFRO syndrome. This report will certainly help to 
clarify the pathogenesis of and optimal treatment strategies for TAFRO syndrome.