WACHOWIAK-J-Białaczki, zespół mielodysplastyczny - Klinika ...
WACHOWIAK-J-Białaczki, zespół mielodysplastyczny - Klinika ...
WACHOWIAK-J-Białaczki, zespół mielodysplastyczny - Klinika ...
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Failure<br />
Suboptimal<br />
Response<br />
Optimal<br />
Response<br />
PAED<br />
II<br />
3 Months<br />
No haematol.<br />
Response<br />
No complete<br />
haematological<br />
remission (CHR)<br />
1 – 2 log reduction<br />
in the<br />
number of BCR-<br />
ABL transcripts<br />
Suttorp-M-EBMT Paed WP Poznan, 2008<br />
Criteria for response assessment of CML<br />
treated with imatinib<br />
Duration of imatinib therapy<br />
6 Months<br />
> 95 % Ph+<br />
35 – 95 % Ph+<br />
< 35 % Ph+<br />
12 Months<br />
> 35 % Ph+<br />
1 – 35 % Ph+<br />
< 0 % Ph+ (CCyR)<br />
< 3 log reduction<br />
in the<br />
number of BCR-<br />
ABL transcripts<br />
18 Months<br />
< 35 % – 1 % Ph+<br />
0 % Ph+ (CCyR)<br />
< 3 log reduction<br />
in the<br />
number of BCR-<br />
ABL transcripts<br />
0 % Ph+ (CCyR)<br />
> = 3 log reduction<br />
in the<br />
number of BCR-<br />
ABL transcripts<br />
No additional cytogenetic aberration except Ph+ !<br />
Stem cell transplantation for chronic myeloid leukemia in children<br />
Cwynarski K, Roberts IAG, Iacobelli S i wsp.<br />
w imieniu EBMT PDs WP oraz EBMT CL WP<br />
Blood 2003; 102: 1224-1231<br />
95 ośrodków<br />
EBMT<br />
OKRES LICZBA<br />
DZIECI<br />
MSD 1985-2001 182<br />
MUD 1985-2001 132<br />
OKRES<br />
OBSERWACJI<br />
(mediana)<br />
1 – 202<br />
miesiące<br />
(49 mies.)<br />
1 – 193<br />
miesiące<br />
(21 mies.)<br />
pLFS<br />
po 3 latach<br />
CP1 63%<br />
AP 35%<br />
CP1 56%<br />
AP 34%<br />
pOS<br />
po 3 latach<br />
CP1 75%<br />
AP 46%<br />
CP1 65%<br />
AP 39%<br />
CML in<br />
chronic phase<br />
CML in advanced<br />
phase (AC / BC)<br />
medium / high dose<br />
imatinib up-front<br />
primary<br />
failure of<br />
imatinib<br />
ALL- or AMLbased<br />
induction<br />
therapy<br />
PAED<br />
II<br />
response<br />
Algorithms for combining Imatinib and SCT in pediatrics<br />
standard dose<br />
imatinib up-front<br />
primary failure<br />
of imatinib<br />
!!! Monitoring !!!<br />
ongoing<br />
response<br />
maximal duration of treatment<br />
2 years<br />
yes<br />
perfectly HLA-matched donor<br />
(either sibling or unrelated;<br />
9-10/10 alleles, high res. typing)<br />
urgent stem cell transplantation<br />
accepting also partially HLAmatched<br />
donors<br />
no<br />
secondary failure<br />
of imatinib<br />
recommend stem<br />
cell transplantation<br />
continue imatinib as<br />
long as effective<br />
Interferon-alpha or 2nd<br />
generation TK-inhibitor<br />
Suttorp-M-EBMT Paed WP Poznan, 2.- 4. 06. 2008<br />
ZESPÓŁ<br />
MIELODYSPLASTYCZNY<br />
(myelodysplastic syndrome, MDS)