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Table 2 Clinical features of TAFRO syndrome in eight previously reported cases and in our case

From: The first report of adolescent TAFRO syndrome, a unique clinicopathologic variant of multicentric Castleman’s disease

Case no. Age/sex Thrombocytopenia (PLT ×104/μl) Anasarca Reticulin fibrosis of the bone marrow Renal dysfunction Organomegaly Hb (g/dl) IgG (mg/dl) Serum IL-6 (pg/ml) (normal range) Plasma VEGF (pg/ml) (normal range) Lymph node biopsy Treatment Outcome Ref
1 47/F 1.5 + + No data + 10.9 1046 No data 285 (<115) No data CHOP, PSL Survival [6]
2 56/M 1.9 + + + + 10.7 863 7.2 (<4) 31 (<115) No data PSL, IVIG, CyA Survival
3 49/M 1.0 + + No data + 11.7 1057 64.9 (<4) 104 (<115) HV-type of CD PSL, IVIG Death (MOF)
4 49/F 1.7 + + + + 6.2 2611 11 (No data) 330 (No data) unclear DEX, PSL, CyA Survival [2]
5 43/F <3.0 + No data + + 12.6 965 45.6 (<4) 665 (<115) HV-type of CD mPSL, PSL, RTX, TCZ Survival [3]
6 47/F 3.9 + + + + 9.1 1426 21.9 (No data) No data PC-type of CD PSL, mPSL, TCZ Survival [4]
7 57/F 1.3 + - + + 7.1 1860 65.5 (No data) 91 (No data) unclear mPSL, PSL, CHOEP Death (Sepsis) [5]
8 73/M 2.4 + + + + 7.6 7080 49 (No data) 25 (No data) Mixed type of CD mPSL, PSL Death (MOF)
Current case 15/M 3.0 + + + + 7.2 729 29 (<3) 178 (<38.3) Mixed type of CD mPSL, PSL, TCZ, IVIG Survival  
  1. CD: Castleman’s disease; CHOEP: cyclophosphamide, doxorubicin, vincristine, etoposide and prednisolone; CHOP: cyclophosphamide, doxorubicin, vincristine and prednisolone; CyA: cyclosporine A; Hb: hemoglobin; HV: hyaline vascular type; IgG: immunoglobulin G; IL-6: interleukin-6; IVIG: intravenous immunoglobulin; MOF: multiple organ failure; mPSL: methylprednisolone; PC: plasma cell type; PLT: platelet; PSL: prednisolone; RTX: rituximab; TCZ: tocilizumab; VEGF: vascular endothelial cell growth factor; Ref: reference number.