TAFRO syndrome is a systemic inflammatory disorder manifesting as thrombocytopenia; anasarca including pleural effusion and ascites; fever; renal insufficiency; and organomegaly including hepatosplenomegaly and lymphadenopathy. Its onset may be acute or sub-acute, but its etiology remains unknown. Although several clinical and pathological characteristics of TAFRO syndrome resemble those of Castleman's disease, other specific features can differentiate between the two. Some patients have been successfully treated with glucocorticoids and/or immunosuppressants including cyclosporin A, tocilizumab and rituximab, whereas others are refractory to treatment, eventually succumbing to the disease. Early and reliable diagnoses and early treatments with appropriate agents are essential to enhancing patient survival. The 2015 updated diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, as formulated by Japanese research teams, are presented herein. Furthermore, clinicopathological data on 28 patients with this condition and similar symptoms (e.g., MCD with serositis and thrombocytopenia) were analyzed retrospectively.