Primary and secondary thrombocytosis in childhood

Br J Haematol. 2005 Apr;129(2):165-77. doi: 10.1111/j.1365-2141.2004.05329.x.


This review summarizes current data on the pathomechanisms and clinical aspects of primary and secondary thrombocytosis in childhood. Primary thrombocytosis is extremely rare in childhood, mostly diagnosed at the beginning of the second decade of life. As in adults, the criteria of the Polycythemia Vera Group are appropriate to diagnose primary thrombocytosis. The pathomechansims of non-familial forms are complex and include spontaneous formation of megakaryopoietic progenitors and increased sensitivity to thrombopoietin (Tpo). Familial forms can be caused by mutations in Tpo or Tpo receptor (c-mpl) genes. These mutations result in overexpression of Tpo, sustained intracellular signalling or disturbed regulation of circulating Tpo. Treatment of primary thrombocytosis is not recommended if platelet counts are <1500/nl and bleeding or thrombosis did not occur in patient's history. In severe cases, decision on treatment should weigh potential risks of treatment options (hydroxyurea, anagrelide) against expected benefits for preventing thrombosis or haemorrhage. Secondary thrombocytosis is frequent in children, in particular in the first decade of life. Hepatic Tpo production is stimulated in acute response reaction to a variety of disorders. Thrombosis prophylaxis is not required, even at platelet counts >1000/nl, except for cases with additional prothrombotic risk factors.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adolescent
  • Bacterial Infections / complications
  • Blood Platelets / physiology
  • Child
  • Child, Preschool
  • Humans
  • Hydroxyurea / therapeutic use
  • Infant
  • Infant, Newborn
  • Liver / metabolism
  • Megakaryocytes / physiology
  • Mutation
  • Nucleic Acid Synthesis Inhibitors / therapeutic use
  • Platelet Aggregation Inhibitors / therapeutic use
  • Platelet Count
  • Quinazolines / therapeutic use
  • Thrombocytosis / diagnosis*
  • Thrombocytosis / drug therapy
  • Thrombocytosis / etiology
  • Thrombopoiesis
  • Thrombopoietin / genetics
  • Thrombopoietin / metabolism
  • Virus Diseases / complications


  • Nucleic Acid Synthesis Inhibitors
  • Platelet Aggregation Inhibitors
  • Quinazolines
  • Thrombopoietin
  • anagrelide
  • Hydroxyurea