Baseline characteristics and posttransfusion Hb decrease
| Case . | Sex . | Age, y . | Diagnosis . | DAT results . | Type of AIHA∗ . | RBC units transfused† . | Hb, g/dL ‡ . | LDH, U/L ‡ . | Bilirubin total, μmol/L ‡ . | Posttransfusion Hb decrease <1 g/dL per 24 hr . | |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Before enrollment (without C1-INH) § . | On trial (with C1-INH) ‖ . | ||||||||||
| 1 | F | 45 | CAS | IgG: 4+ | Mixed | 2 | 5.6 | 975 | 65 | Yes | Yes |
| APS | IgM: 2+ | ||||||||||
| IgA: 2+ | |||||||||||
| C3d: 3+ | |||||||||||
| 2 | F | 37 | CAS | IgG:4+, IgM: 2+, | Mixed | 3 | 3.7 | 1118 | 152 | No | No |
| IBD | IgA: neg | ||||||||||
| T-cell NHL, allo-SCT | C3d: 3+ | ||||||||||
| 3 | F | 38 | CAS | IgG: 4+ | Mixed | 7 | 3.5 | 911 | 55 | No data | No |
| SLE | IgM: 4+, | ||||||||||
| RA | IgA: 3+ | ||||||||||
| IBD | C3d: 4+ | ||||||||||
| ITP | |||||||||||
| 4 | M | 69 | CAS | IgG: 4+ | Mixed | 2 | 5.3 | 559 | 36 | No data | Yes |
| B-cell NHL | IgM: neg | ||||||||||
| IgA: neg | |||||||||||
| C3d: 3+ | |||||||||||
| 5 | M | 59 | CAD | IgG: neg IgM: neg IgA: neg C3d: 2+ | Cold | 1 | 4.8 | 605 | 30 | Yes | Yes |
| 6 | F | 46 | CAS, allo-SCT, MF | IgG: 2+ IgM: neg IgA: neg C3d: 3+ | Mixed | 23 | 5.3 | 270 | 53 | N/A | Yes |
| 7 | M | 65 | CAS, CLL | IgG: neg¶ IgM: 1+ IgA: neg C3d: 3+ | Cold | 8 | 5.5 | 526 | 96 | No | No |
| 8 | M | 72 | CAS, | IgG: neg | Cold | 2 | 6.2 | 476 | 13 | Yes | Yes |
| CLL | IgM: neg | ||||||||||
| IgA: neg | |||||||||||
| C3d: 4+ | |||||||||||
| 9 | F | 77 | CAD | IgG: neg | Cold | 4 | 6.9 | 570 | 26 | Yes | Yes |
| IgA: neg | |||||||||||
| IgM: 2+ | |||||||||||
| C3d: 4+ | |||||||||||
| 10 | M | 75 | CAD | IgG: neg | Cold | 1 | 6.9 | 495 | 55 | Yes | Yes |
| IgM: neg | |||||||||||
| IgA: neg | |||||||||||
| C3d: 3+ | |||||||||||
| Median | 62 | 2.5 | 5.4 | 564.5 | 54 | ||||||
| Response/no. of patients | 5/7 | 7/10 | |||||||||
| Case . | Sex . | Age, y . | Diagnosis . | DAT results . | Type of AIHA∗ . | RBC units transfused† . | Hb, g/dL ‡ . | LDH, U/L ‡ . | Bilirubin total, μmol/L ‡ . | Posttransfusion Hb decrease <1 g/dL per 24 hr . | |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Before enrollment (without C1-INH) § . | On trial (with C1-INH) ‖ . | ||||||||||
| 1 | F | 45 | CAS | IgG: 4+ | Mixed | 2 | 5.6 | 975 | 65 | Yes | Yes |
| APS | IgM: 2+ | ||||||||||
| IgA: 2+ | |||||||||||
| C3d: 3+ | |||||||||||
| 2 | F | 37 | CAS | IgG:4+, IgM: 2+, | Mixed | 3 | 3.7 | 1118 | 152 | No | No |
| IBD | IgA: neg | ||||||||||
| T-cell NHL, allo-SCT | C3d: 3+ | ||||||||||
| 3 | F | 38 | CAS | IgG: 4+ | Mixed | 7 | 3.5 | 911 | 55 | No data | No |
| SLE | IgM: 4+, | ||||||||||
| RA | IgA: 3+ | ||||||||||
| IBD | C3d: 4+ | ||||||||||
| ITP | |||||||||||
| 4 | M | 69 | CAS | IgG: 4+ | Mixed | 2 | 5.3 | 559 | 36 | No data | Yes |
| B-cell NHL | IgM: neg | ||||||||||
| IgA: neg | |||||||||||
| C3d: 3+ | |||||||||||
| 5 | M | 59 | CAD | IgG: neg IgM: neg IgA: neg C3d: 2+ | Cold | 1 | 4.8 | 605 | 30 | Yes | Yes |
| 6 | F | 46 | CAS, allo-SCT, MF | IgG: 2+ IgM: neg IgA: neg C3d: 3+ | Mixed | 23 | 5.3 | 270 | 53 | N/A | Yes |
| 7 | M | 65 | CAS, CLL | IgG: neg¶ IgM: 1+ IgA: neg C3d: 3+ | Cold | 8 | 5.5 | 526 | 96 | No | No |
| 8 | M | 72 | CAS, | IgG: neg | Cold | 2 | 6.2 | 476 | 13 | Yes | Yes |
| CLL | IgM: neg | ||||||||||
| IgA: neg | |||||||||||
| C3d: 4+ | |||||||||||
| 9 | F | 77 | CAD | IgG: neg | Cold | 4 | 6.9 | 570 | 26 | Yes | Yes |
| IgA: neg | |||||||||||
| IgM: 2+ | |||||||||||
| C3d: 4+ | |||||||||||
| 10 | M | 75 | CAD | IgG: neg | Cold | 1 | 6.9 | 495 | 55 | Yes | Yes |
| IgM: neg | |||||||||||
| IgA: neg | |||||||||||
| C3d: 3+ | |||||||||||
| Median | 62 | 2.5 | 5.4 | 564.5 | 54 | ||||||
| Response/no. of patients | 5/7 | 7/10 | |||||||||
Eight out of 10 patients received either a prophylactic or therapeutic dose of anticoagulation.
allo-SCT, allogeneic stem cell transplantation; APS, antiphospholipid syndrome; CAS, cold agglutinin syndrome; CLL, chronic lymphocytic lymphoma; F, female; IBD, inflammatory bowel disease; ITP, immune thrombocytopenia; neg, negative; NHL, non-Hodgkin lymphoma; M, male; MF, myelofibrosis; RA, rheumatoid arthritis; SLE, systemic lupus erythematosus.
AIHA classified as warm AIHA: DAT positive for IgG and/or IgA ± C3d when a clinically significant cold-reactive antibody has been excluded; cold AIHA: monospecific DAT strongly positive for C3d (and negative or weakly positive with IgG) and CAs are present; mixed AIHA: diagnosed in patients with a DAT positive for C3d and IgG DAT, a CA with a thermal amplitude ≥30 °C, and evidence of a warm IgG antibody by eluate.
Total in the 30 days before inclusion.
Normal values: Hb male, 13.2 to 16.6 g/dL; Hb female, 11.6 to 15 g/dL; LDH, 0 to 247 U/L; total bilirubin, 0 to 17 μmol.
Post hoc analysis (see “Methods”).
Per protocol analysis.
IgG weakly positive in DAT, No evidence for IgG reacting autoantibodies in the eluate.