E2A-PBX1 fusion in adult acute lymphoblastic leukaemia: biological and clinical features.
Molecular and cytogenetic studies performed in 305 adult acute lymphoblastic leukaemia (ALL) patients enrolled in the gimema (Gruppo Italiano Malattie EMatologiche dell'Adulto) multicentric protocols identified an E2A-PBX1 fusion and/or t(1;19) in 10 patients (3.3%). All had common ALL, were mostly CyIg+ and were CD34/CD13/CD33-. Nine patients achieved a complete remission (CR); five patients showed a haematological relapse after 7 months (median). Four patients are alive in first CR with a median follow-up of 29 months; three patients are molecularly negative. This abnormality is frequently associated with early treatment failure. E2A-PBX1+ adult ALL should be considered for intensified treatment strategies and monitoring of minimal residual disease.
Foa
Robin
R
Dipartimento di Biotecnologie Cellulari ed Ematologia, University La Sapienza, Rome, Italy. rfoa@bce.uniroma1.it
Vitale
Antonella
A
Mancini
Marco
M
Cuneo
Antonio
A
Mecucci
Cristina
C
Elia
Loredana
L
Lombardo
Romina
R
Saglio
Giuseppe
G
Torelli
Giuseppe
G
Annino
Luciana
L
Specchia
Giorgina
G
Damasio
Eugenio
E
Recchia
Anna
A
Di Raimondo
Francesco
F
Morra
Enrica
E
Volpe
Ettore
E
Tafuri
Agostino
A
Fazi
Paola
P
Hunger
Stephen P
SP
Mandelli
Franco
F
D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002878:Chromosomes, Human, Pair 1 / Q000235:genetics; D002888:Chromosomes, Human, Pair 19 / Q000235:genetics; D005260:Female; D005500:Follow-Up Studies; D018398:Homeodomain Proteins / Q000235:genetics*; D006801:Humans; D007621:Karyotyping; D008297:Male; D008875:Middle Aged; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy / Q000235:genetics*; D011379:Prognosis; D012008:Recurrence; D014178:Translocation, Genetic; D017211:Treatment Failure
D018398:Homeodomain Proteins; D015514:Oncogene Proteins, Fusion; C089847:E2A-Pbx1 fusion protein