Leukemia Research Reports (Jan 2021)

Pediatric M5 acute myeloid leukemia with MLL-SEPT6 fusion and a favorable outcome

  • Alain Chebly,
  • Claudia Djambas Khayat,
  • Tony Yammine,
  • Rima Korban,
  • Warde Semaan,
  • Jessica Bou Zeid,
  • Chantal Farra

Journal volume & issue
Vol. 16
p. 100277

Abstract

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Acute myeloid leukemia (AML) patients with MLL-SEPT6 fusion represent a small subset of AML. The uncommon MLL-SEPT6 rearrangement results from t(X;11) or other variants like ins(X;11), and it is usually associated with complex cytogenetic abnormalities. We herein report a case of AML-M5-infant with ins(X;11)(q24;q23q13) and MLL-SEPT6. The one-year-old boy presented with leukocytosis, anemia and thrombocytopenia. He had a favorable response to chemotherapy according to ELAM02protocol and is currently in complete remission. We here, highlight the occurrence of MLL-SEPT6 as the sole abnormality in a pediatric-AML-M5 case, discuss the prognostic implication of this genetic variant, while reviewing previously reported AML-MLL-SEPT6 cases.

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