2019 Updated diagnostic criteria and disease severity classification for TAFRO syndrome

A pediatric case of tocilizumab‐resistant TAFRO syndrome treated successfully with rituximab

“…Light microscopy and immunostaining of the cervical lymph node revealed atrophy of germinal centers, proliferation of high endothelial venules with filtration of mature plasma cells, and HHV‐8 and Epstein Barr virus negativity (Supplemental Figure S1). The symptoms and findings in this patient were all consistent with the diagnostic criteria of Iwaki et al 4 and Masaki et al's 5 for TAFRO syndrome, with a severity score of 8 5 . Therefore, we started treatments with methylprednisolone pulse therapy, tocilizumab, and cyclosporine (Figure 1).…”

A Case of Repeated TAFRO Syndrome-Like Symptoms and Retroperitoneal Hemorrhage in a Patient With Sjögren Syndrome

“…The symptoms in this present case closely resembled TAFRO syndrome in terms of clinicopathological findings, aggressive clinical course, and beneficial effects of tocilizumab and rituximab [1][2][6][7]12]. However, according to the diagnostic criteria for TAFRO syndrome proposed by Masaki et al, TAFRO syndrome can only be diagnosed after excluding Sjögren syndrome, and the thrombocytopenia, anasarca, fever, myelofibrosis, organomegaly, and renal insufficiency in this patient can be accounted for by Sjögren syndrome [2,11,[13][14].…”

“…Considering that a diagnosis of TAFRO syndrome requires the exclusion of Sjögren syndrome and the absence of hypergammaglobulinemia [2,8], we concluded that his disease was a Sjögren syndrome with TAFRO syndrome-like symptoms. Although cytomegalovirus IgM was positive on admission, the intravenous administration of ganciclovir was ineffective.…”

“…Its diagnostic criteria, disease severity classification, and treatment strategy were proposed by Masaki et al in 2015 [1]. According to the updated 2019 diagnostic criteria for TAFRO syndrome, Sjögren syndrome has been newly added to the exclusion criteria during differential diagnosis [2]. However, patients with a definitive diagnosis of Sjögren syndrome or those positive for Sjögren's antibodies but with an uncertain diagnosis have reportedly manifested the clinicopathological features of TAFRO syndrome [3][4][5][6][7].…”

Frontline Siltuximab and Rituximab in TAFRO syndrome: A case report

“…Thrombocytopenia, Anasarca, reticulin Fibrosis of the bone marrow, Renal dysfunction and organomegaly (TAFRO) syndrome is a very rare and severe variant of multicenter Castleman's disease firstly reported in Japan in 2010 1,2 . TAFRO syndrome is a hyper‐cytokine disease often caused by an uncontrolled HHV‐8 replication with a severe reduction of T follicular helper cells and atrophic lymphoid follicles; however, histology is not a mandatory diagnostic criterion for TAFRO 3 ; therefore, a prompt start of immunosuppressive and anti‐cytokine therapy is allowed.…”