Table 1: Summary of the most common treatment regimens for those diagnosed with TAFRO syndrome.

Case reportAge (years)/genderTAFRO-directed treatmentGeneral outcome
1st line2nd line3rd line

Simons, 2016 22/MTCZTCZ, rituximab, etoposideClinical improvement

Tedesco, 201521/FPrednisone, TCZRituximab, cyclophosphamide, vincristine, prednisoneClinical improvement

Takai et al., 2010 [17] 39/MMethylprednisolone, prednisoloneClinical improvement
38/MPrednisoloneClinical improvement

Koduri et al., 2014 [15]14/MCHOP 6ThalidomidePersistent lymphadenopathy, no edema or ascites

Kubokawa et al., 2014 [7]15/MMethylprednisoloneTCZ, IVIGCytokine levels decreased by 4 months

Ozawa et al., 2014 [10]48/FIVIGSteroidsRituximabResolution of thrombocytopenia and marrow fibrosis

Kawabata et al., 2013 [9]47/FPrednisoneTCZ, steroidsImprovement of IL-6 levels, no signs of disease recurrence

Inoue et al., 2013 [8]49/FDexamethasoneCyclosporine AResolution of thrombocytopenia and marrow fibrosis

Iwaki et al., 2013 [11]43/FMethylprednisoloneRituximab, TCZ, steroidsImprovement of thrombocytopenia and anasarca

Masaki et al., 2013 [12] 57/FMethylprednisoloneCHOEPDied from septic shock
73/MPrednisoloneDied from multiorgan failure

Takai et al., 2010 [17]47/FCHOP, prednisolonePrednisolone (maintenance)Clinical improvement
56/MMethylprednisolone, IVIGCyclosporin AImproved platelet count, edema, ascites
49/MMethylprednisolone, IVIGDied from multiorgan failure
53/FPrednisolonePrednisolone, cyclophosphamideClinical improvement
56/FMethylprednisolone, cyclosporin AClinical improvement

Abdo et al., 2014 [18]81/MPrednisoneClinical improvement