No | Centre | Age at disease start (years) | Age at ASCT4-150(years) | TCD4-150(Y/N) | Conditioning | Follow up (months) | Outcome | Present antirheumatic drugs | ||||||||
1–11 | Utrecht, NL | 1–7 | 7–14 | CD2/3 (6) | Cy4-150, ATG4-150, TBI4-150 (4 Gy) | 4–36 | 7 remission, 1partial remission, 1 fatal MAS4-150 | Low steroids (2) | ||||||||
NSAIDs (1) | ||||||||||||||||
12–14 | Leiden, NL | 2–10 | 4, 6, 14 | CD2/3 (1) | Cy, ATG, TBI (4 Gy) | 12–15 | 1 remission, 1 partial remission, 1 fatal MAS | Salazopyrin (1) | ||||||||
CD34 (2) | ||||||||||||||||
15 | Paris, Fr | 8 | 9 | CD34 | Cy, ATG | — | Day 18 fatal MAS | |||||||||
16–17 | Goteborg, Swe | 4–9 | CD2/3 | Cy, ATG, TBI (4 Gy) | 3–10 | Both remission | Low steroids (1) | |||||||||
18–22 | Triest, It | 3–15 | 8–20 | VCR | Cy, ALG (3) | 12–36 | Remission (3) | Etanercept (1), | ||||||||
Flu4-150, ALG4-150(2) | Relapse (2) | NSAIDs (1) | ||||||||||||||
23 | Portland US | 2 | 10 | CD34 | Cy, ATG, TBI (4Gy) | 7 | Complete remission | None | ||||||||
24–25 | Brussels, Be | 1–7 | 8, 17 | CD34 | Cy, ATG | 7–19 | Complete remission | None | ||||||||
26 | Brussels, Be | 15 | CD34 | Cy, ATG | — | Fatal cardiac toxicity | — | |||||||||
27–28 | Osaka, JP | 1–5 | 3–8 | CD34 | CY, ATG (1) | 4–11 | No response (1) | Low steroids | ||||||||
VP16, TT4-150(1) | Remission (1) | None | ||||||||||||||
29 | Newcastle, UK | 3 | 10 | CD34 | Cy, ATG | 2 | Remission | Low steroids | ||||||||
30 | Slowakije | 18 | 35 | None | Bu4-150, Cy, ATG | 6 | Partial remission | Low steroids | ||||||||
31 | Newcastle, UK | 21 | 23 | Campath | Cy, Campath | 2 | Amyloidosis, septic shock, no MAS | |||||||||
32 | Ferrara, It | 33 | 35 | CD34 (PSCM) | Cy, ATG | 18 | ATG related toxicity, Complete remission | Low steroids |
↵4-150 SCT = stem cell transplantation; JIA = juvenile idiopathic arthritis; ASCT = autologous stem cell transplant; TCD = T cell depletion; Cy = cyclophosphamide; ATG = anti-thymocyte globulin; TBI = total body irradiation; ALG = anti-lymphocyte globulin; Flu= fludarabine; TT = thiotepa; Bu = busulfan; MAS = macrophage activation syndrome; NSAIDs = non-steroidal anti-inflammatory drugs.