Distribution of NPM1-ALK and X-ALK Fusion Transcripts in Paediatric Anaplastic Large Cell Lymphoma: A Molecular-Histological Correlation

Br J Haematol. 2009 Aug;146(3):306-9. doi: 10.1111/j.1365-2141.2009.07754.x. Epub 2009 May 26.

Abstract

Anaplastic large cell lymphomas (ALCL) in children express anaplastic lymphoma kinase (ALK) fusion genes, most commonly NPM1-ALK. The distribution of X-ALK among 66 childhood ALCLs was analysed. One ALCL was ALK-negative. Reverse transcription polymerase chain reaction detected NPM1-ALK in 58 tumours, all showing nuclear and cytoplasmic ALK staining. The remaining seven ALCL stained for ALK in the cytoplasm only: two expressed TPM3-ALK, one ATIC-ALK, one MYH9-ALK; three no TPM3-, TFG-, ATIC-, CLTC- or MYH9-ALK. Almost 90% of paediatric ALK-positive ALCLs express NPM1-ALK. There was complete concordance between ALK staining pattern and the presence of a typical/variant ALK fusion partner.

MeSH terms

  • Adolescent
  • Anaplastic Lymphoma Kinase
  • Child
  • Child, Preschool
  • Female
  • Humans
  • In Situ Hybridization, Fluorescence
  • Infant
  • Lymphoma, Large-Cell, Anaplastic / genetics*
  • Lymphoma, Large-Cell, Anaplastic / metabolism
  • Male
  • Molecular Motor Proteins / metabolism
  • Myosin Heavy Chains / metabolism
  • Oncogene Proteins, Fusion / metabolism*
  • Protein-Tyrosine Kinases / genetics*
  • Protein-Tyrosine Kinases / metabolism
  • Receptor Protein-Tyrosine Kinases
  • Reverse Transcriptase Polymerase Chain Reaction
  • Tropomyosin / metabolism

Substances

  • ATIC-ALK fusion protein, human
  • MYH9 protein, human
  • Molecular Motor Proteins
  • Oncogene Proteins, Fusion
  • TPM3 protein, human
  • Tropomyosin
  • oncoprotein CLTCL-ALK
  • p80(NPM-ALK) protein
  • ALK protein, human
  • Anaplastic Lymphoma Kinase
  • Protein-Tyrosine Kinases
  • Receptor Protein-Tyrosine Kinases
  • Myosin Heavy Chains