B-cell acute lymphoblastic leukemia with mature phenotype and MLL rearrangement: report of five new cases and review of the literature

Elisa Olga Sajaroff, Adrian Mansini, Patricia Rubio, Cristina Noemí Alonso, Marta S. Gallego, Mariela C. Coccé, Silvia Eandi-Eberle, Andrea Raquel Bernasconi, Maria Ampatzidou, George Paterakis, Stefanos I. Papadhimitriou, Loizos Petrikkos, Vassilios Papadakis, Sophia Polychronopoulou, Jorge G. Rossi, Maria Sara Felice

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2 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)2289-2297
Number of pages9
JournalLeukemia and Lymphoma
Volume57
Issue number10
DOIs
StatePublished - Oct 2 2016

Keywords

  • Acute lymphoblastic leukemia
  • MLL
  • mature phenotype

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