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Paediatric B-cell precursor acute lymphoblastic leukaemia with t(1;19)(q23;p13): clinical and cytogenetic characteristics of 47 cases from the Nordic countries treated according to NOPHO protocols.

Artikel i vetenskaplig tidskrift
Författare Mette K Andersen
Kirsi Autio
Gisela Barbany
Georg Borgström
Lucia Cavelier
Irina Golovleva
Sverre Heim
Kristina Heinonen
Randi Hovland
Johann H Johannsson
Bertil Johansson
Eigil Kjeldsen
Ann Nordgren
Lars Palmqvist
Erik Forestier
Publicerad i British journal of haematology
Volym 155
Nummer/häfte 2
Sidor 235–243
ISSN 1365-2141
Publiceringsår 2011
Publicerad vid Institutionen för biomedicin, avdelningen för klinisk kemi och transfusionsmedicin
Sidor 235–243
Språk en
Länkar dx.doi.org/10.1111/j.1365-2141.2011...
Ämnesord acute lymphoblastic leukaemia; paediatric; t(1;19); TCF3; PBX1
Ämneskategorier Medicinsk genetik, Molekylär medicin (genetik och patologi), Hematologi

Sammanfattning

The translocation t(1;19)(q23;p13)/der(19)t(1;19) is a risk stratifying aberration in childhood B-cell precursor acute lymphoblastic leukaemia (BCP ALL) in the Nordic countries. We have identified 47 children/adolescents with t(1;19)/der(19)t(1;19)-positive BCP ALL treated on two successive Nordic Society of Paediatric Haematology and Oncology (NOPHO) protocols between 1992 and 2007 and have reviewed the clinical and cytogenetic characteristics of these cases, comprising 1·8% of all cases. The translocation was balanced in 15 cases (32%) and unbalanced in 29 cases (62%). The most common additional chromosome abnormalities were del(9p), i(9q), del(6q), and del(13q). The median age was 7 years, the median white blood cell (WBC) count was 16 × 10(9) /l, and the female/male ratio was 1·2. The predicted event-free survival (EFS) at 5 and 10 years was 0·79, whereas the predicted overall survival (OS) at 5 and 10 years was 0·85 and 0·82, respectively. Nine patients had a bone marrow relapse after a median of 23 months; no patient had a central nervous system relapse. Additional cytogenetic abnormalities, age, gender, WBC count or whether the t(1;19) was balanced or unbalanced did not influence EFS or OS. Compared to cases with t(12,21) and high hyperdiploidy, EFS was similar, but overall survival was worse in patients with t(1;19)/der(19)t(1;19) (P = 0·004).

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