Myeloproliferative Neoplasms in Children and Adolescents and Thrombosis at Unusual Sites: The Role of Driver Mutations. Issue 6 (August 2019)

• Record Type: Journal Article
• Authors: Tafesh, Laith; Musgrave, Kathryn; Roberts, Wing; Plews, Dianne; Carey, Peter; Biss, Tina
• Is Part Of: Journal of pediatric hematology/oncology. Volume 41:Issue 6(2019)
• Publication Date: 2019-08
• Subjects: myeloproliferative disorders -- pediatrics -- polycythemia vera -- thrombocythemia essential -- thrombosis; Pediatric hematology -- Periodicals; Tumors in children -- Periodicals; 618.9215
• DOI: 10.1097/MPH.0000000000001173
• Languages: English
• ISSNs: 1077-4114

Abstract

Abstract : Myeloproliferative neoplasms (MPNs) in childhood and adolescence are rare and seldom complicated by thrombosis. We describe 3 cases of thrombosis at unusual sites in young patients with MPNs. In the pediatric MPN population, unlike in adult MPNs, a clonal mutation is identifiable in only a minority of cases (22% to 26%). All 3 of these individuals had JAK2 mutations driving the disease process. A literature search identified 19 cases of MPN-associated thrombosis in children. Seventeen of the 19 children (89.5%) had a driver mutation. These cases suggest that identifiable driver mutations may confer an increased thrombotic risk in children with MPNs.