Recently, a unique clinicopathologic variant of multicentric Castleman's disease 
(MCD) has been identified in Japan. This disease is characterized by a 
constellation of symptoms, as listed in the title, and multiple lymphadenopathy 
of mild degree with a pathologic diagnosis of atypical CD, often posing 
diagnostic and therapeutic problems for pathologists and hematologists, 
respectively. These findings suggest that this disease represents a novel 
clinical entity belonging to systemic inflammatory disorders with a background 
of immunological abnormality beyond the ordinal spectrum of MCD. To define this 
disorder more clearly, Japanese participants presented clinicopathologic data at 
the Fukushima and Nagoya meetings. Many of the patients presented by the 
participants were significantly accompanied by a combination of 
thrombocytopenia, ascites (anasarca), pleural effusions, microcytic anemia, 
fever, myelofibrosis, renal dysfunction, and organomegaly (TAFRO). Multiple 
lymphadenopathies were generally of mild degree, less than 1.5 cm in diameter, 
and consistently featured the histopathology of mixed- or less hyaline 
vascular-type CD. Autoantibodies were often detected. However, this disease did 
not fulfill the diagnostic criteria for well-known autoimmune diseases including 
systemic lupus erythematosus. Castleman-Kojima disease and TAFRO syndrome (the 
favored clinical term) were proposed for this disease. The patients were 
sensitive to steroid and anti-interleukin-6 receptor antibody (tocilizumab), but 
some exhibited a deteriorated clinical course despite the treatment. The 
participants proposed a future nationwide survey and a Japanese consortium to 
facilitate further clinical and therapeutic studies of this novel disease. [J 
Clin Exp Hematop 53(1): 57-61, 2013].