Table 2.
Baseline characteristics for patients included in meta-analysis.
| Variable | Flore et al. (n = 12) | Prajwal et al. (n = 9) | Stephan et al. (n = 15) | Joslyn et al. (n = 10) | Michelle et al.(n = 6) | Sonata et al. (n = 64) |
|---|---|---|---|---|---|---|
| Age, median (range) | 39(1.2–66) | 7(2–61) | 48(23–66) | 44(17–59) | 5.2(2.5–25) | 5.5(2.7–11.7) |
| Gender, male (%) | 7(58) | 6(67) | 7(47) | 4(40) | 4(67) | 40(63) |
| Primary disease | Hematological disease | Hematological disease/others | Hematological disease | Hematological disease | Hematological disease/others | Hematological disease/others |
| Type of transplant | Allo/UCB | Allo/Auto | Allo | Allo/UCB | Auto | Allo/Auto/UCB |
| Conditioning regimen | MAC/RIC | MAC/RIC | MAC/RIC | MAC/RIC | MAC | MAC/RIC |
| Other risk factors at diagnosis, number (%) | ||||||
| CNI used | 8(67) | 7(78) | 9(60) | 10(100) | NA | 49(77) |
| aGvHD | 8(67) | 5(56) | 12(80) | 7(70) | 0(0) | 14(22) |
| Affection | 6(50) | 2(22) | 8(67) | 8(80) | 1(17) | 6(9) |
| Interval between HSCT and diagnosis, median days | 121 | 68 | 264 | 93 | 35 | <100a |
| sC5b-9 | NA | NA | 456(127-810) | NA | 151.5(100-460) | 398(282-544) |
| Interval between diagnosis and Eculizumab therapy, median days | 31 | 24 | 10 | 4 | 18 | NA |
| Eculizumab therapy, median days | 65 | 178 | 52.5 | 48.5 | 110 | 66 |
| First-line therapy, number (%)/second-line therapy, number | 5(42)/7 | 2(22)/7 | 11(73)/4 | 7(70)/3 | 6(100)/0 | 64(100)/0 |
| Eculizumab dose, median dose | 6 | 8 | 9 | 6 | 9.5 | 11 |
| Overall response, number (%) | 6(50) | 7(78) | 13(93)b | 7(70) | 4(67) | 41(64) |
| Complete response, number (%) | 2(17) | 5(56) | NA | 1(10) | 1(17) | 36(56) |
| Survivals, number (%) | 4(33) | 7(78) | 5(33) | 6(60) | 4(67) | 35(55) |
| Median follow-up months | 14 | 12 | 8 | 13 | 30 | 15 |
| AEs during Eculizumab therapy | Infection | No | Infection | Skin rash | NA | Infection |
| Cause of death, numbers (%) | ||||||
| TA-TMA related | 4(50) | 0 | 2(20) | 0 | 1(50) | 8(28) |
| Infection | 2(25) | 0 | 8(80) | 2(50) | 0 | 6(21) |
| GvHD | 2(25) | 2(100) | 0 | 1(25) | 0 | 14(48) |
| Relapse of the primary disease | 0 | 0 | 0 | 1(25) | 1(50) | 1(3) |
| Prognosis | CKD | CKD | CKD | CKD | CKD/HTN | CKD/HTN |
Allo,; Auto, Autologous HSCT; CNI, calcineurin inhibitors; HTN hypertension; MAC, myeloablative regimen; RIC, reduced intensity regimen; UCB, umbilical cord blood.
a
As 92% patients were diagnosed TA-TMA at a median of 23 days (IQR 3–48), and five had TA-TMA between 118 and 221 days after transplant, we regarded that the median days of interval between HSCT and diagnosis was less than 100 days.
b
one unknown response due to early death.