Suggested sequence of treatments in primary and secondary WAIHA and CAIHA
| Disease or condition . | First line . | Second line . | Beyond second line . | Last resort . | References . |
|---|---|---|---|---|---|
| Primary AIHA | Steroids | Splenectomy rituximab | Azathioprine, MMF, cyclosporin, cyclophosphamide | High-dose cyclophosphamide, alemtuzumab | See text |
| B- and T-cell NHL | Steroids | Chemotherapy ± rituximab (splenectomy in SMZL) | 71,72 | ||
| Hodgkin lymphoma | Steroids | Chemotherapy (radiotherapy) | 20 | ||
| Solid tumors | Steroids, surgery | 21 | |||
| Ovarian dermoid cyst* | Ovariectomy | 22 | |||
| SLE | Steroids | Azathioprine | MMF | Rituximab autologous SCT | See text |
| Ulcerative colitis | Steroids | Azathioprine | Total colectomy | 24,73 | |
| CVID | Steroids + IgG | Splenectomy | 25 | ||
| ALPD* | Steroids | MMF | Sirolimus | 74,75 | |
| Allogeneic SCT | Steroids | Rituximab | Splenectomy, T-cell infusion | 76 | |
| Organ transplantation (pancreas)* | Discontinuation of immune suppression, steroids | Splenectomy | 28 | ||
| Interferon α | Withdrawal | Steroids | |||
| Primary CAD | Protection from cold exposure | Rituximab, chlorambucil | Eculizumab,† bortezomib† | 64,77,–79 | |
| Paroxysmal cold hemoglobinuria | Supportive treatment | Rituximab | 80 |
| Disease or condition . | First line . | Second line . | Beyond second line . | Last resort . | References . |
|---|---|---|---|---|---|
| Primary AIHA | Steroids | Splenectomy rituximab | Azathioprine, MMF, cyclosporin, cyclophosphamide | High-dose cyclophosphamide, alemtuzumab | See text |
| B- and T-cell NHL | Steroids | Chemotherapy ± rituximab (splenectomy in SMZL) | 71,72 | ||
| Hodgkin lymphoma | Steroids | Chemotherapy (radiotherapy) | 20 | ||
| Solid tumors | Steroids, surgery | 21 | |||
| Ovarian dermoid cyst* | Ovariectomy | 22 | |||
| SLE | Steroids | Azathioprine | MMF | Rituximab autologous SCT | See text |
| Ulcerative colitis | Steroids | Azathioprine | Total colectomy | 24,73 | |
| CVID | Steroids + IgG | Splenectomy | 25 | ||
| ALPD* | Steroids | MMF | Sirolimus | 74,75 | |
| Allogeneic SCT | Steroids | Rituximab | Splenectomy, T-cell infusion | 76 | |
| Organ transplantation (pancreas)* | Discontinuation of immune suppression, steroids | Splenectomy | 28 | ||
| Interferon α | Withdrawal | Steroids | |||
| Primary CAD | Protection from cold exposure | Rituximab, chlorambucil | Eculizumab,† bortezomib† | 64,77,–79 | |
| Paroxysmal cold hemoglobinuria | Supportive treatment | Rituximab | 80 |
MMF indicates mycophenolate mofetil; NHL, non-Hodgkin lymphoma; SMZL, splenic marginal zone lymphoma; SLE, systemic lupus erythematosus; SCT, stem cell transplantation; CVID, common variable immunodeficiency; ALPD, autoimmune lymphoproliferative disease; and CAD, cold agglutinin disease.
No personal experience.
Off-label use.