CD10- pre-B acute lymphoblastic leukemia (ALL) is a distinct high-risk subgroup of adult ALL associated with a high frequency of MLL aberrations: results of the German Multicenter Trials for Adult ALL (GMALL).
Immunophenotyping disclosed CD10 negativity in 70 of 2408 cases of B-lineage acute lymphoblastic leukemia (ALL), although other criteria followed classification of pre-B ALL (eg, cytoplasmic immunoglobulin positivity). These blasts showed high myeloid antigen expression (60% CD65 positivity) and reacted with antibody 7.1 in 95% of the cases. MLL-AF4 fusion transcripts or an 11q23/MLL rearrangement or both were evident in 46 of 56 samples (82%). Although 83% of the patients achieved complete remission, the remission duration remained remarkably low: 141 days for MLL rearrangement-positive and 245 days for MLL rearrangement-negative CD10(-) pre-B ALL. Thus, the overall survival probability 3 years after diagnosis was 0.34 +/- 0.20 SE in MLL-rearrangement-negative versus 0.12 +/- 0.06 SE in MLL rearrangement-positive CD10- pre-B ALL. Our data identify CD10- cytoplasmic immunoglobulin-positive pre-B ALL as a rare (2.2%) but distinct immuno-subtype of adult ALL that is characterized by a high MLL rearrangement rate and a worse outcome.
Gleissner
Beate
B
Department of Internal Medicine III, Campus Benjamin Franklin, Charité Universitätsmedizin, Hindenburgdamm 30, 12200 Berlin, Germany. beate.gleissner@charite.de
Goekbuget
Nicola
N
Rieder
Harald
H
Arnold
Renate
R
Schwartz
Stefan
S
Diedrich
Helmut
H
Schoch
Claudia
C
Heinze
Barbara
B
Fonatsch
Christa
C
Bartram
Claus R
CR
Hoelzer
Dieter
D
Thiel
Eckhard
E
D000293:Adolescent; D000328:Adult; D000368:Aged; D002869:Chromosome Aberrations; D002880:Chromosomes, Human, Pair 11 / Q000235:genetics; D005260:Female; D005858:Germany; D006801:Humans; D008297:Male; D008875:Middle Aged; D015260:Neprilysin / Q000378:metabolism*; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000235:genetics* / Q000473:pathology* / Q000628:therapy; D012307:Risk Factors; D015996:Survival Rate; D014158:Transcription, Genetic / Q000235:genetics; D016896:Treatment Outcome
D015260:Neprilysin