Successful treatment of a child with T/myeloid acute bilineal leukemia associated with TLX3/BCL11B fusion and 9q deletion

Pediatr Blood Cancer. 2011 Mar;56(3):467-9. doi: 10.1002/pbc.22850. Epub 2010 Nov 11.

Abstract

Acute bilineal leukemias are rare and are commonly associated with t(9;22) and MLL abnormalities. Herein, we report a pediatric case of bilineal T/myeloid acute leukemia associated with del (9q)(q13q22) and TLX3/BCL11B fusion due to the cryptic t(5;14)(q35;32). FISH studies confirmed the TLX3/BCL11B fusion in both the myeloid and lymphoid blasts, while the 9q deletion was restricted to the lymphoid component. Optimal therapy for such patients remains controversial and it is not clear if they should be treated with ALL or AML-based chemotherapeutic regimens. Our patient has been in extended remission following ALL-based chemotherapy and a matched unrelated cord blood transplant. Inc.

Publication types

  • Case Reports

MeSH terms

  • Antineoplastic Agents / therapeutic use
  • Bone Marrow Transplantation
  • Child
  • Chromosomes, Human, Pair 22 / genetics
  • Chromosomes, Human, Pair 9 / genetics*
  • Combined Modality Therapy
  • Homeodomain Proteins / genetics*
  • Humans
  • In Situ Hybridization, Fluorescence
  • Leukemia, Biphenotypic, Acute / diagnosis
  • Leukemia, Biphenotypic, Acute / genetics
  • Leukemia, Biphenotypic, Acute / therapy*
  • Leukemia, Myeloid, Acute / diagnosis
  • Leukemia, Myeloid, Acute / genetics
  • Leukemia, Myeloid, Acute / therapy*
  • Leukemia, T-Cell / diagnosis
  • Leukemia, T-Cell / genetics
  • Leukemia, T-Cell / therapy*
  • Male
  • Oncogene Proteins, Fusion / genetics*
  • RNA, Messenger / genetics
  • Remission Induction
  • Repressor Proteins / genetics*
  • Reverse Transcriptase Polymerase Chain Reaction
  • Sequence Deletion
  • Translocation, Genetic / genetics
  • Treatment Outcome
  • Tumor Suppressor Proteins / genetics*

Substances

  • Antineoplastic Agents
  • BCL11B protein, human
  • Homeodomain Proteins
  • Oncogene Proteins, Fusion
  • RNA, Messenger
  • Repressor Proteins
  • TLX3 protein, human
  • Tumor Suppressor Proteins