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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)

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