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

Author information

Affiliations

  • 1. Department of Immunology and Rheumatology , Hospital De Pediatría Prof. Dr. Juan P. Garrahan , Buenos Aires , Argentina ;
      Authors
    • Sajaroff EO 1
    • Bernasconi AR 1
    • Rossi JG 1
    (3 authors)
  • 2. Department of Hematology-Oncology , Hospital De Pediatría Prof. Dr. Juan P. Garrahan , Buenos Aires , Argentina ;
      Authors
    • Mansini A 2
    • Rubio P 2
    • Alonso CN 2
    • Eandi-Eberle S 2
    • Felice MS 2
    (5 authors)
  • 3. Cytogenetics Laboratory, Department of Genetics , Hospital De Pediatría Prof. Dr. Juan P. Garrahan , Buenos Aires , Argentina ;
      Authors
    • Gallego MS 3
    • Coccé MC 3
    (2 authors)
  • 4. Department of Pediatric Hematology-Oncology (TAO) , Aghia Sophia ChildreńS Hospital , Athens , Greece ;
      Authors
    • Ampatzidou M 4
    • Petrikkos L 4
    • Papadakis V 4
    • Polychronopoulou S 4
    (4 authors)
  • 5. Department of Flow Cytometry, Laboratory of Immunology , "G. Gennimatas" General Hospital , Athens , Greece ;
      Authors
    • Paterakis G 5
    (1 author)
    • Show all (6)

Leukemia & Lymphoma, 08 Feb 2016, 57(10):2289-2297
https://doi.org/10.3109/10428194.2016.1141407 PMID: 26857438 

Review

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.

Full text links 

Read article at publisher's site: https://doi.org/10.3109/10428194.2016.1141407

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