Baseline characteristics
| . | Age∗/ sex . | AIHA, DAT results . | Associated condition . | Acrocyanosis . | Previous AIHA treatments . | Ongoing immune suppressive treatments at start daratumumab . | Hb, g/dL, at start daratumumab . | Bilirubin total, μmmol/L, at start daratumumab . | LDH, U/L, at start daratumumab . | RBC transfusion in month before start daratumumab . |
|---|---|---|---|---|---|---|---|---|---|---|
| 1. | 37/F | wAIHA DAT >2+ IgG | - | - | Steroids, rituximab splenectomy, azathioprine, cyclophosphamide bortezomib, mycophenolate, sirolimus, cyclosporin | Steroids | 11.2 | 53 | 425 | - |
| 2. | 54/F | wAIHA, DAT IgG>2+ and IgA>2+ | - | - | Steroids, rituximab EPO, mycophenolate, cyclosporin | Steroids | 9.7 | 32 | 584 | - |
| 3 | 53/M | wAIHA, DAT IgG>2+ | Myasthenia gravis | - | Steroids, rituximab, EPO, azathioprine, mycophenolate, cyclosporin, bortezomib | Steroids | 10 | 74 | 914 | - |
| 4 | 56/F | wAIHA DAT>2+ IgG | SLE | - | Steroids, mycophenolate, rituximab, IVIG EPO, cyclophosphamide,bortezomib | Steroids, hydroxychloro-quine EPO | 10.5 | 11 | 288 | - |
| 5 | 53/F | wAIHA DAT IgG>2+ and C>2+ | - | - | Steroids, rituximab splenectomy IVIG, EPO, azathioprine, bortezomib, sirolimus, cyclosporin, tacrolimus | Steroids, IVIG maintenance (monthly) | 8.8 | 86 | 242 | - |
| 6† | 55/F | wAIHA DAT IgG>2+ and C<2+ | Evans syndrome | - | Steroids, rituximab, splenectomy, azathioprine, bortezomib, everolimus, cyclosporin | - | 8.2 | 31 | 1100 | 10 |
| 7† | 55/F | wAIHA DAT IgG>2+ and C<2+ | Evans syndrome | - | Steroids, rituximab | Steroids | 10.7 | 24 | 335 | - |
| 8 | 42/M | wAIHA DAT IgG>2+ and C<2+ | Evans syndrome, MGUS IgG kappa Nonclonal CD8 T-cell proliferation, T-LGL suspect | - | Steroids, rituximab , splenectomy, plasmapheresis, cyclophosphamide, sirolimus, cyclosporin, danazole, methotrexate | Steroids Sirolimus Plasmapheresis | 4 | 140 | 4800 | 30 |
| 9 | 25/F | wAIHA DAT IgG>2+ | Evans syndrome | - | Steroids, rituximab, IVIG, bortezomib, sirolimus, danazol, EPO | Danazol Sirolimus | 6.3 | 19 | 421 | - |
| 10 | 59/M | wAIHA DAT>2+ IgG | IgG MGUS | - | Steroids, rituximab IVIG, EPO, splenectomy | Steroids Rituximab, IVIG, EPO, splenectomy | 6.1 | 80 | 1609 | 36 |
| 11 | 36/F | wAIHA DAT<2+ IgG | SLE | - | Steroids, rituximab, Obinutuzumab, plasmapheresis, azathioprine, ibrutinib | Steroids IVIG | 7.8 | 41 | 282 | 10 |
| 12 | 64/F | wAIHA DAT IgG <2+ | Evans syndrome, IgG kappa and lambda MGUS | - | Steroid, rituximab, IVIG | Steroids | 7.3 | 61.2 | 671 | 1 |
| 13† | 59/M | cAIHA, DAT negative Low titer cold agglutinin present | CAD IgG kappa MGUS, HBV | Yes | Steroids, rituximab EPO, bortezomib | - | 7 | 67 | 518 | 2 |
| 14 | 62/M | cAIHA DAT C>2+ | CAD | - | Steroids, rituximab, IVIG, plasmapheresis, eculizumab | Steroids Eculizumab | 8.8 | 73 | 480 | 20 |
| 15† | 56/M | cAIHA DAT IgG<2+, IgM>2+ | LPL | Yes | Steroids, rituximab, cyclophosphamide, bortezomib, lenalidomide | - | 12.5‡ | 10 | 351 | - |
| 16 | 74/M | cAIHA DAT C<2+ | CAD | Yes | Rituximab, plasmapheresis | - | 11.2 | 12 | - | - |
| 17 | 80/F | cAIHA DAT C>2+ | LPL | Yes | Steroids, rituximab, cyclophosphamide, mycophenolate, ibrutinib | Steroids | 8.5 | 46 | 972 | - |
| 18 | 75/F | cAIHA | CAD | Yes | Steroids, rituximab, mycophenolate, azathioprine | - | 8.6§ | 57 | 809 | 1 |
| 19 | 70/M | cAIHA | CAD, type 1 cryoglobuli-naemia | Yes | Steroids, plasmapheresis 9 doses of pegcetacoplan/ placebo‖, 1 dose of BIVV020¶ | - | 5 | 30 | 1330 | 4 |
| Median (range) | 56 (25-80) | 5 (2-10) | 8.6 (4-12.5) | 46 (10-140) | 584 (242-4800) | 10 (1-36) |
| . | Age∗/ sex . | AIHA, DAT results . | Associated condition . | Acrocyanosis . | Previous AIHA treatments . | Ongoing immune suppressive treatments at start daratumumab . | Hb, g/dL, at start daratumumab . | Bilirubin total, μmmol/L, at start daratumumab . | LDH, U/L, at start daratumumab . | RBC transfusion in month before start daratumumab . |
|---|---|---|---|---|---|---|---|---|---|---|
| 1. | 37/F | wAIHA DAT >2+ IgG | - | - | Steroids, rituximab splenectomy, azathioprine, cyclophosphamide bortezomib, mycophenolate, sirolimus, cyclosporin | Steroids | 11.2 | 53 | 425 | - |
| 2. | 54/F | wAIHA, DAT IgG>2+ and IgA>2+ | - | - | Steroids, rituximab EPO, mycophenolate, cyclosporin | Steroids | 9.7 | 32 | 584 | - |
| 3 | 53/M | wAIHA, DAT IgG>2+ | Myasthenia gravis | - | Steroids, rituximab, EPO, azathioprine, mycophenolate, cyclosporin, bortezomib | Steroids | 10 | 74 | 914 | - |
| 4 | 56/F | wAIHA DAT>2+ IgG | SLE | - | Steroids, mycophenolate, rituximab, IVIG EPO, cyclophosphamide,bortezomib | Steroids, hydroxychloro-quine EPO | 10.5 | 11 | 288 | - |
| 5 | 53/F | wAIHA DAT IgG>2+ and C>2+ | - | - | Steroids, rituximab splenectomy IVIG, EPO, azathioprine, bortezomib, sirolimus, cyclosporin, tacrolimus | Steroids, IVIG maintenance (monthly) | 8.8 | 86 | 242 | - |
| 6† | 55/F | wAIHA DAT IgG>2+ and C<2+ | Evans syndrome | - | Steroids, rituximab, splenectomy, azathioprine, bortezomib, everolimus, cyclosporin | - | 8.2 | 31 | 1100 | 10 |
| 7† | 55/F | wAIHA DAT IgG>2+ and C<2+ | Evans syndrome | - | Steroids, rituximab | Steroids | 10.7 | 24 | 335 | - |
| 8 | 42/M | wAIHA DAT IgG>2+ and C<2+ | Evans syndrome, MGUS IgG kappa Nonclonal CD8 T-cell proliferation, T-LGL suspect | - | Steroids, rituximab , splenectomy, plasmapheresis, cyclophosphamide, sirolimus, cyclosporin, danazole, methotrexate | Steroids Sirolimus Plasmapheresis | 4 | 140 | 4800 | 30 |
| 9 | 25/F | wAIHA DAT IgG>2+ | Evans syndrome | - | Steroids, rituximab, IVIG, bortezomib, sirolimus, danazol, EPO | Danazol Sirolimus | 6.3 | 19 | 421 | - |
| 10 | 59/M | wAIHA DAT>2+ IgG | IgG MGUS | - | Steroids, rituximab IVIG, EPO, splenectomy | Steroids Rituximab, IVIG, EPO, splenectomy | 6.1 | 80 | 1609 | 36 |
| 11 | 36/F | wAIHA DAT<2+ IgG | SLE | - | Steroids, rituximab, Obinutuzumab, plasmapheresis, azathioprine, ibrutinib | Steroids IVIG | 7.8 | 41 | 282 | 10 |
| 12 | 64/F | wAIHA DAT IgG <2+ | Evans syndrome, IgG kappa and lambda MGUS | - | Steroid, rituximab, IVIG | Steroids | 7.3 | 61.2 | 671 | 1 |
| 13† | 59/M | cAIHA, DAT negative Low titer cold agglutinin present | CAD IgG kappa MGUS, HBV | Yes | Steroids, rituximab EPO, bortezomib | - | 7 | 67 | 518 | 2 |
| 14 | 62/M | cAIHA DAT C>2+ | CAD | - | Steroids, rituximab, IVIG, plasmapheresis, eculizumab | Steroids Eculizumab | 8.8 | 73 | 480 | 20 |
| 15† | 56/M | cAIHA DAT IgG<2+, IgM>2+ | LPL | Yes | Steroids, rituximab, cyclophosphamide, bortezomib, lenalidomide | - | 12.5‡ | 10 | 351 | - |
| 16 | 74/M | cAIHA DAT C<2+ | CAD | Yes | Rituximab, plasmapheresis | - | 11.2 | 12 | - | - |
| 17 | 80/F | cAIHA DAT C>2+ | LPL | Yes | Steroids, rituximab, cyclophosphamide, mycophenolate, ibrutinib | Steroids | 8.5 | 46 | 972 | - |
| 18 | 75/F | cAIHA | CAD | Yes | Steroids, rituximab, mycophenolate, azathioprine | - | 8.6§ | 57 | 809 | 1 |
| 19 | 70/M | cAIHA | CAD, type 1 cryoglobuli-naemia | Yes | Steroids, plasmapheresis 9 doses of pegcetacoplan/ placebo‖, 1 dose of BIVV020¶ | - | 5 | 30 | 1330 | 4 |
| Median (range) | 56 (25-80) | 5 (2-10) | 8.6 (4-12.5) | 46 (10-140) | 584 (242-4800) | 10 (1-36) |
EPO, erythropoietin; HBV, hepatitis B virus; HCV, hepatitis C virus; IVIG, intravenous immune globulin; MGUS, monoclonal gammopathy of unknown significance.
Age in years at start daratumumab.
Previously published.
Treatment indication was severe acrocyanosis.
After RBC transfusion.
Participated in the randomized, placebo-controlled phase 3 trial trial (ClinicalTrials.gov, NCT05096403).
Participated in the PDY16370 study, received 1 dose of BIVV020 (anti-C1s humanized IgG4 monoclonal antibody), but went off study (ClinicalTrials.gov, NCT04269551).