A family with hereditary thrombocythaemia and normal genes for thrombopoietin and c-Mpl

Br J Haematol. 2006 Nov;135(3):348-51. doi: 10.1111/j.1365-2141.2006.06316.x. Epub 2006 Sep 22.

Abstract

Hereditary thrombocythaemia (HT) is an inherited autosomal dominant disorder. Recent studies reported six different mutations, four within the thrombopoietin (TPO) gene and two within c-Mpl (TPO receptor) gene in six unrelated families with HT. This study investigated the molecular basis of hereditary thrombocythaemia in an Israeli-Jewish family. We screened the genes for TPO and c-Mpl by amplification and sequencing of all the corresponding exons including exon/intron boundaries and promoters. In addition, plasma levels of TPO and erythropoietin (EPO) were measured. No abnormality in the TPO/c-Mpl genes has been identified in affected HT family members. Plasma TPO and EPO levels were found to be normal/low or normal respectively in the individuals affected. In conclusion, lack of a molecular lesion within either TPO or cMpl genes indicate that HT may be caused by factors other than TPO-cMpl axis in this family.

MeSH terms

  • Adolescent
  • Adult
  • Child, Preschool
  • DNA / genetics
  • Erythropoietin / blood
  • Family Health*
  • Female
  • Humans
  • Male
  • Middle Aged
  • Pedigree
  • Receptors, Thrombopoietin / genetics*
  • Sequence Analysis, DNA / methods
  • Thrombocytosis / blood
  • Thrombocytosis / genetics*
  • Thrombopoietin / blood
  • Thrombopoietin / genetics*

Substances

  • Receptors, Thrombopoietin
  • Erythropoietin
  • MPL protein, human
  • DNA
  • Thrombopoietin