Early T-Cell Precursor Acute Lymphoblastic Leukemia in an Infant With an NRAS Q61R Mutation and Clinical Features of Juvenile Myelomonocytic Leukemia

Pediatr Blood Cancer. 2016 Sep;63(9):1667-70. doi: 10.1002/pbc.26050. Epub 2016 May 4.

Abstract

Early T-cell precursor acute lymphoblastic leukemia (ETP-ALL) is a subtype of T-acute lymphoblastic leukemia (T-ALL) arising from a primitive precursor. We present a unique case of an infant with ETP-ALL with a missense NRAS mutation in codon 61 (c.182A>G, p.Q61R). The patient also had a minor population of non-ETP T-ALL blasts and clinical features typically associated with juvenile myelomonocytic leukemia (JMML), namely, absolute monocytosis, splenomegaly, and elevated hemoglobin F. The treatment was initiated with chemotherapy, followed by cord blood transplantation. The patient achieved remission, but unfortunately died from transplant-related complications. This case highlights an NRAS mutation in ETP-ALL with JMML-like phenotype.

Keywords: ETP-ALL; JMML; NRAS.

Publication types

  • Case Reports

MeSH terms

  • Cord Blood Stem Cell Transplantation
  • GTP Phosphohydrolases / genetics*
  • Humans
  • Infant
  • Leukemia, Myelomonocytic, Juvenile / genetics*
  • Leukemia, Myelomonocytic, Juvenile / therapy
  • Male
  • Membrane Proteins / genetics*
  • Mutation, Missense*
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / genetics*
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / therapy

Substances

  • Membrane Proteins
  • GTP Phosphohydrolases
  • NRAS protein, human