Current and new therapies for wAIHA secondary to CLL
| Treatment . | Line . | ORR, % . | Comments . | Ref. . |
|---|---|---|---|---|
| Prednisone: 1 mg/kg per day for 3-4 wk | First | 84-90 | Response rates comparable with those reported for primary wAIHA, but relapse risk is higher | 30,31 |
| Dexamethasone: 40 mg/d for 4 d, 2-6 cycles every 2-4 wk | First | 100 | Response rates comparable with those reported for primary wAIHA, but relapse risk is higher | 30,31 |
| Rituximab: 375 mg/m2 weekly for 4 wk | Second or > | 72-80 | Single agent in patients with indolent CLL; if ineffective, CLL therapy should be pursued | 32,33 |
| RCD | Second or > | 81-100 | Small series of relapsed/refractory wAIHAs (17 patients) | 34,35 |
| R-CVP | Second or > | 95 | Small series of relapsed/refractory wAIHAs (17 patients) | 36,37 |
| Rituximab + bendamustine | Second or > | 81 | 26 patients with progressive CLL, and active wAIHA | 38 |
| Alemtuzumab: 30 mg × 3/wk for 4-12 wk | Third or > | 100 | Small series of relapsed/refractory wAIHAs and progressive CLL (8 patients) | 39,40 |
| Cyclosporine: 3-5 mg/kg per day | Third or > | 56 | Small series of relapsed/refractory wAIHAs (16 patients) | 41 |
| Splenectomy | Third or > | 69-78 | Old reports and small series; splenectomy is currently discouraged | 42 |
| Ofatumumab: Cycle 1: day 1, 300 mg; and day 8, 1000 mg; further cycles 1000 mg on day 1, monthly | n/a | n/a | Case report | 43 |
| Obinutuzumab: Cycle 1: day 1 100 mg; and day 2, 900 mg; further cycles 1000 mg on day 1, monthly | n/a | n/a | Case report | 44 |
| Ibrutinib: 420 mg/d | n/a | n/a | Case reports: phase 2 trial ongoing | 45,46 |
| Venetoclax: 200-400 mg/d | n/a | n/a | Case reports | 47 |
| Treatment . | Line . | ORR, % . | Comments . | Ref. . |
|---|---|---|---|---|
| Prednisone: 1 mg/kg per day for 3-4 wk | First | 84-90 | Response rates comparable with those reported for primary wAIHA, but relapse risk is higher | 30,31 |
| Dexamethasone: 40 mg/d for 4 d, 2-6 cycles every 2-4 wk | First | 100 | Response rates comparable with those reported for primary wAIHA, but relapse risk is higher | 30,31 |
| Rituximab: 375 mg/m2 weekly for 4 wk | Second or > | 72-80 | Single agent in patients with indolent CLL; if ineffective, CLL therapy should be pursued | 32,33 |
| RCD | Second or > | 81-100 | Small series of relapsed/refractory wAIHAs (17 patients) | 34,35 |
| R-CVP | Second or > | 95 | Small series of relapsed/refractory wAIHAs (17 patients) | 36,37 |
| Rituximab + bendamustine | Second or > | 81 | 26 patients with progressive CLL, and active wAIHA | 38 |
| Alemtuzumab: 30 mg × 3/wk for 4-12 wk | Third or > | 100 | Small series of relapsed/refractory wAIHAs and progressive CLL (8 patients) | 39,40 |
| Cyclosporine: 3-5 mg/kg per day | Third or > | 56 | Small series of relapsed/refractory wAIHAs (16 patients) | 41 |
| Splenectomy | Third or > | 69-78 | Old reports and small series; splenectomy is currently discouraged | 42 |
| Ofatumumab: Cycle 1: day 1, 300 mg; and day 8, 1000 mg; further cycles 1000 mg on day 1, monthly | n/a | n/a | Case report | 43 |
| Obinutuzumab: Cycle 1: day 1 100 mg; and day 2, 900 mg; further cycles 1000 mg on day 1, monthly | n/a | n/a | Case report | 44 |
| Ibrutinib: 420 mg/d | n/a | n/a | Case reports: phase 2 trial ongoing | 45,46 |
| Venetoclax: 200-400 mg/d | n/a | n/a | Case reports | 47 |
n/a, not applicable; ORR, overall response rate; RCD, rituximab plus cyclophosphamide and dexamethasone; R-CVP, rituximab plus cyclophosphamide, vincristine, and prednisone.