Multicentric Castleman's disease (MCD) is a polyclonal lymphoproliferative 
disorder that manifests as marked hyper-γ-globulinemia, severe inflammation, 
anemia, and thrombocytosis. Recently, Takai et al. reported a new disease 
concept, TAFRO syndrome, named from thrombocytopenia, anasarca, fever, reticulin 
fibrosis, and organomegaly. Furthermore, Kojima et al. reported Japanese MCD 
cases with effusion and thrombocytopenia (Castleman-Kojima disease). Here, we 
report two cases of MCD associated with marked pleural effusion, ascites, and 
thrombocytopenia, and discuss the independence of the TAFRO syndrome 
(Castleman-Kojima disease). Case 1: A 57-year-old woman had fever, anemia, 
anasarca, and some small cervical lymphadenopathy. Although she had been 
administered steroid therapy, and full-coverage antibiotics, her general 
condition, including fever, systemic inflammation, and anasarca, deteriorated 
steadily. We administered chemotherapy [CHOEP (cyclophosphamide, doxorubicin, 
vincristine, etoposide, and prednisolone) regimen], but despite a transient 
improvement, she died due to septic shock. Case 2: A 73-year-old man with a 
history of aplastic anemia and remission presented with fever, severe 
inflammation, and anasarca. Prednisolone was administered (15 mg daily), and his 
hyperinflammation once improved. Nevertheless, his general condition, including 
pleural effusion and ascites, worsened, and C-reactive protein and interleukin-6 
levels showed marked increases. The patient died due to multiorgan failure. 
Cases of TAFRO syndrome (Castleman-Kojima disease) are still rare. Therefore, it 
is necessary to conduct multicenter clinical surveys including similar cases, 
such as ours, to reach a consensus regarding diagnostic criteria, therapeutic 
strategy, and pathophysiological etiology for this syndrome.