Table 3.

iMCD-TAFRO cases

TherapyPatients (n)Response (%)No response (%)Treatment failure (%)
All therapies 49 65/98 (66) 33/98 (34) 52/98 (53) 
Corticosteroid monotherapy 25 9/25 (36) 16/25 (64) 18/25 (72) 
Cyclophosphamide-based cytotoxic chemotherapy 14 13/14 (93) 1/14 (7) 4/14 (29) 
Rituximab with cytotoxic agent 1/1 (100) 0/1 (0) 0/1 (0) 
Rituximab without cytotoxic agent 10 9/10 (90) 1/10 (10) 4/10 (40) 
Cytotoxic regimen (without cyclophosphamide or rituximab) 2/3 (67) 1/3 (33) 1/3 (33) 
Tocilizumab with or without steroids 20 15/20 (75) 5/20 (25) 10/20 (50) 
Siltuximab with or without steroids 1/1 (100) 0/1 (0) 1/1 (100) 
Cyclosporine A (without cytotoxic agent) 6/8 (75) 2/8 (25) 2/8 (25) 
Immunomodulators: other than cyclosporine A (without cytotoxic agent) 5/9 (56) 4/9 (44) 5/9 (56) 
Other 4/7 (57) 3/7 (43) 7/7 (100) 
TherapyPatients (n)Response (%)No response (%)Treatment failure (%)
All therapies 49 65/98 (66) 33/98 (34) 52/98 (53) 
Corticosteroid monotherapy 25 9/25 (36) 16/25 (64) 18/25 (72) 
Cyclophosphamide-based cytotoxic chemotherapy 14 13/14 (93) 1/14 (7) 4/14 (29) 
Rituximab with cytotoxic agent 1/1 (100) 0/1 (0) 0/1 (0) 
Rituximab without cytotoxic agent 10 9/10 (90) 1/10 (10) 4/10 (40) 
Cytotoxic regimen (without cyclophosphamide or rituximab) 2/3 (67) 1/3 (33) 1/3 (33) 
Tocilizumab with or without steroids 20 15/20 (75) 5/20 (25) 10/20 (50) 
Siltuximab with or without steroids 1/1 (100) 0/1 (0) 1/1 (100) 
Cyclosporine A (without cytotoxic agent) 6/8 (75) 2/8 (25) 2/8 (25) 
Immunomodulators: other than cyclosporine A (without cytotoxic agent) 5/9 (56) 4/9 (44) 5/9 (56) 
Other 4/7 (57) 3/7 (43) 7/7 (100) 

These were compiled from published case reports and small series.

n, number of subjects treated in each treatment regimen category, with a total of 98 regimens evaluated. Other includes plasma exchange (n = 3), plasma exchange + corticosteroids (n = 2), polymyxin B–immobilized fiber column direct hemoperfusion and cytokine absorption (n = 1), allogeneic stem cell transplant (n = 1). Please refer to supplemental appendix 1 for complete list of references.

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