In:
British Journal of Haematology, Wiley, Vol. 149, No. 1 ( 2010-04), p. 84-92
Abstract:
Acute leukaemias of ambiguous lineage (ALAL) represent a rare type of leukaemia, expressing both myeloid and lymphoid markers. This study retrospectively analyzed data from 92 children (biphenotypic n = 78, bilineal n = 6, lineage switch n = 8) with ALAL registered in the Berlin‐Frankfürt‐Münster (BFM) acute myeloid leukaemia (AML) and acute lymphoblastic leukaemia (ALL) studies between 1998 and 2006 (2·4% of all cases with acute leukaemia). Our cohort of ALAL patients was characterized by comparatively high median age (8·9 years), high median white blood cell count (14·9 × 10 9 /l), as well as frequent hyperleucocytosis (18·5%) and central nervous system involvement (24·1%). The most frequent cytogenetic abnormalities were ETV6/RUNX1 fusion (16%) and trisomy 8 (14·6%). Complete remission rate was significantly lower than in ALL‐BFM patients (91·8% vs. 99·1%, P 〈 0·001), but comparable to AML‐BFM patients (87·9%). Event‐free survival (EFS) and overall survival (OS) of ALAL patients were low, at 62 ± 5%. 5‐year probability of EFS was significantly worse than in ALL patients (80 ± 1%, P 〈 0·001), but better than for AML patients (49 ± 2%, P = 0·027). Our data suggest that an intensive therapy regimen including stem cell transplantation may be favourable for bilineal or lineage switch cases, whereas patients with ETV6/RUNX1 fusion, lymphoid morphology and patients with expression of cyCD22 and cyCD79a should be treated with an ALL‐directed therapy.
Type of Medium:
Online Resource
ISSN:
0007-1048
,
1365-2141
DOI:
10.1111/bjh.2010.149.issue-1
DOI:
10.1111/j.1365-2141.2009.08058.x
Language:
English
Publisher:
Wiley
Publication Date:
2010
detail.hit.zdb_id:
80077-6
detail.hit.zdb_id:
1475751-5
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