iMCD clinical case series of 344 patients
| Therapy . | Patients (n) . | Response/m* (%) . | No response/m* (%) . | Treatment failure/m* (%) . | Data combined from references . |
|---|---|---|---|---|---|
| All therapies | 344 | 281/461 (61) | 180/461 (39) | 163/367 (44) | 11,21,32,33,39, supplementary appendix citations |
| Corticosteroid monotherapy | 117 | 53/114 (46) | 61/114 (54) | 62/115 (54) | 22,23,44, supplementary appendix citations |
| Corticosteroid or cytotoxic chemotherapy (not distinguished) | 19 | 12/19 (63) | 7/19 (37) | NA | 21 |
| Cytotoxic chemotherapy (any time used) | 135 | 102/131 (78) | 29/131 (22) | 44/105 (42) | 7,22,23, supplementary appendix citations |
| Anti–IL-6 mAb (without cytotoxic agent or rituximab) | 147 | 88/144 (61) | 56/144 (39) | 32/100 (32) | 7,22,23,43,44, supplementary appendix citations |
| Immunomodulator (without cytotoxic agent) | 27 | 18/26 (69) | 8/26 (31) | 10/26 (38) | 23, supplementary appendix citations |
| Other | 16 | 8/13 (62) | 5/13 (38) | 12/15 (80) | 23, supplementary appendix citations |
| No treatment/follow-up only | 18 | 0/14 (0) | 14/14 (100) | 11/14 (79) | 7,22,23, supplementary appendix citations |
| Therapy . | Patients (n) . | Response/m* (%) . | No response/m* (%) . | Treatment failure/m* (%) . | Data combined from references . |
|---|---|---|---|---|---|
| All therapies | 344 | 281/461 (61) | 180/461 (39) | 163/367 (44) | 11,21,32,33,39, supplementary appendix citations |
| Corticosteroid monotherapy | 117 | 53/114 (46) | 61/114 (54) | 62/115 (54) | 22,23,44, supplementary appendix citations |
| Corticosteroid or cytotoxic chemotherapy (not distinguished) | 19 | 12/19 (63) | 7/19 (37) | NA | 21 |
| Cytotoxic chemotherapy (any time used) | 135 | 102/131 (78) | 29/131 (22) | 44/105 (42) | 7,22,23, supplementary appendix citations |
| Anti–IL-6 mAb (without cytotoxic agent or rituximab) | 147 | 88/144 (61) | 56/144 (39) | 32/100 (32) | 7,22,23,43,44, supplementary appendix citations |
| Immunomodulator (without cytotoxic agent) | 27 | 18/26 (69) | 8/26 (31) | 10/26 (38) | 23, supplementary appendix citations |
| Other | 16 | 8/13 (62) | 5/13 (38) | 12/15 (80) | 23, supplementary appendix citations |
| No treatment/follow-up only | 18 | 0/14 (0) | 14/14 (100) | 11/14 (79) | 7,22,23, supplementary appendix citations |
Literature review of published case reports, small series, and clinical trials were compiled to inform and substantiate the experience and opinion of the Working Group authors. Cytotoxic chemotherapy regimens described may include the use of rituximab.
Treatment failure was defined as disease progression while on treatment or insufficient response requiring additional treatments. The main series included in this analysis are referenced. A detailed breakdown of the data is provided in supplemental appendix 1. The TAFRO case reports are tabulated in Table 3.
m, total number of regimens evaluated (479); m*, number regimens assessed for stated outcome; MDACC, MD Anderson Cancer Center case series; n, number of subjects treated in each treatment regimen category. Other includes plasma exchange (n = 4), radiation (n = 2), plasma exchange + corticosteroids (n = 2), IV immunoglobulin (n = 2), Polymyxin B–immobilized fiber column direct hemoperfusion and cytokine absorption (n = 1), allogeneic stem cell transplant (n = 1), Cimetidine (n = 1), antibiotics (n = 1), corticosteroids and etanercept (n = 1), interferon-α (n = 1).