TY - JOUR
T1 - B-cell acute lymphoblastic leukemia with mature phenotype and MLL rearrangement
T2 - report of five new cases and review of the literature
AU - Sajaroff, Elisa Olga
AU - Mansini, Adrian
AU - Rubio, Patricia
AU - Alonso, Cristina Noemí
AU - Gallego, Marta S.
AU - Coccé, Mariela C.
AU - Eandi-Eberle, Silvia
AU - Bernasconi, Andrea Raquel
AU - Ampatzidou, Maria
AU - Paterakis, George
AU - Papadhimitriou, Stefanos I.
AU - Petrikkos, Loizos
AU - Papadakis, Vassilios
AU - Polychronopoulou, Sophia
AU - Rossi, Jorge G.
AU - Felice, Maria Sara
N1 - Publisher Copyright:
© 2016 Informa UK Limited, trading as Taylor & Francis Group.
PY - 2016/10/2
Y1 - 2016/10/2
N2 - The association between mature-B phenotype and MLL abnormalities in acute lymphoblastic leukemia (ALL) is a very unusual finding; only 14 pediatric cases have been reported so far. We describe the clinical and biological characteristics and outcome of five pediatric cases of newly diagnosed B lineage ALL with MLL abnormalities and mature immunophenotype based on light chain restriction and surface Ig expression. Blasts showed variable expression of CD10/CD34/TdT. MLL abnormalities with no MYC involvement were detected in all patients by G-banding, FISH, and/or RT-PCR. Three patients were treated according to Interfant protocol, one to ALLIC-09, and one received B-NHL-BFM-2004. All patients achieved complete remission and three of them relapsed. Despite the small cohort size, it could be postulated that B lineage ALL with MLL abnormalities and mature phenotype is a distinct entity that differs both from the typical Pro B ALL observed in infants and mature B-ALL with high MYC expression.
AB - The association between mature-B phenotype and MLL abnormalities in acute lymphoblastic leukemia (ALL) is a very unusual finding; only 14 pediatric cases have been reported so far. We describe the clinical and biological characteristics and outcome of five pediatric cases of newly diagnosed B lineage ALL with MLL abnormalities and mature immunophenotype based on light chain restriction and surface Ig expression. Blasts showed variable expression of CD10/CD34/TdT. MLL abnormalities with no MYC involvement were detected in all patients by G-banding, FISH, and/or RT-PCR. Three patients were treated according to Interfant protocol, one to ALLIC-09, and one received B-NHL-BFM-2004. All patients achieved complete remission and three of them relapsed. Despite the small cohort size, it could be postulated that B lineage ALL with MLL abnormalities and mature phenotype is a distinct entity that differs both from the typical Pro B ALL observed in infants and mature B-ALL with high MYC expression.
KW - Acute lymphoblastic leukemia
KW - MLL
KW - mature phenotype
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U2 - 10.3109/10428194.2016.1141407
DO - 10.3109/10428194.2016.1141407
M3 - Article
C2 - 26857438
AN - SCOPUS:84958033751
SN - 1042-8194
VL - 57
SP - 2289
EP - 2297
JO - Leukemia and Lymphoma
JF - Leukemia and Lymphoma
IS - 10
ER -
