Sequential change in serum VEGF levels in a case of tocilizumab-resistant TAFRO syndrome treated effectively with rituximab

Abstract

Abstract Recently, a unique clinicopathologic variant of multicentric Castleman disease, TAFRO (i.e. thrombocytopenia, anasarca, fever, renal failure or reticulin fibrosis and organomegaly) syndrome, has been identified in Japan. Previous reports have shown that affected patients usually respond to anti-interleukin 6 (IL-6) receptor antibody, but not all patients achieve remission. Here, we present a 62-year-old man meeting the criteria of TAFRO syndrome. Serum, plasma and ascites levels of cytokines, including IL-6 and vascular endothelial growth factor, were markedly elevated. Tocilizumab, an anti-IL-6 receptor antibody, and corticosteroids were initially used to treat the increase in acute inflammatory proteins and the anasarca, resulting in decreased cytokine levels. However, the patient showed a rapidly progressive course of anasarca and ascites, and an increase in acute inflammatory proteins and cytokine levels shortly thereafter. Rituximab, an anti-CD20 antibody, successfully induced remission of disease symptoms and decreased cytokine levels. The patient was successfully treated with rituximab despite being refractory to tocilizumab and corticosteroids. During our patient’s clinical course, monitoring cytokine profiles, especially vascular endothelial growth factor, was useful in tracking the disease activity of TAFRO syndrome.