Open AccessEssential Thrombocythemia in Children: A Retrospective Study
Author(s) -
Muhammed Ameen,
Khawar Siddiqui,
Saadiya Khan,
Mahasen Saleh,
Abdullah AlJefri,
Abdulrahman Al-Musa
Publication year - 2021
Publication title -
journal of hematology
Language(s) - English
Resource type - Journals
eISSN - 1927-1220
pISSN - 1927-1212
DOI - 10.14740/jh822
Subject(s) - medicine , essential thrombocythemia , anagrelide , pediatrics , thrombocytosis , family history , incidence (geometry) , polycythemia vera , platelet , physics , optics
Essential thrombocythemia (ET) is one of the "classic" Philadelphia chromosome negative (Ph-) myeloproliferative neoplasms characterized by sustained thrombocytosis, increased megakaryopoiesis and high risk of vascular complications. ET is very rare in childhood. The annual incidence is approximately 1 per 10,000,000 in children less than 14 years, and about 60 times lower than adults. The genetic landscape and clonal features in childhood ET has not been well defined. There is no evidence-based guidance on the diagnosis of childhood ET.