Abstract Number: LPB0123
Meeting: ISTH 2021 Congress
Background: Thrombotic events are well-known complications of the BCR-ABL-negative, classical myeloproliferative neoplasms (MPNs). Both arterial and venous thromboses can occur, especially splanchnic vein thromboses (including Budd-Chiari syndrome). There are various potential risk factors, including JAK2V617F, the most common MPN driver mutation, yet height of platelet count has been shown to not correlate with thrombosis risk. While older age is an established risk factor, thrombotic events have been reported in pediatric MPN patients, but the available literature is limited.
Aims: To describe the thrombotic events in a pediatric MPN cohort.
Methods: We reviewed charts of our pediatric MPN cohort for data around thrombotic events, which included demographic information, type of thrombotic event, blood counts at time of event, and mutational status. All subjects are enrolled on an IRB-approved observational study where consent/assent was obtained.
Results: In a cohort of 42 children with MPNs, 6 (14.3%) had a thrombotic event. Five subjects were female, and five were adolescents when thrombosis developed. Five subjects had a JAK2V617F mutation, while one was negative for any known mutation. One subject had a stroke, one had a cerebral venous sinus thrombosis and pulmonary embolism, and four had Budd-Chiari syndrome. Two subjects developed their thrombotic events after diagnosis of their MPN, while four subjects’ thrombotic events occurred at MPN presentation. Five subjects had thrombocytosis (but none had extreme thrombocytosis – platelets >1000×109/L), four had polycythemia, and four had leukocytosis at the time of their thrombotic events.
Conclusions: Our data confirms the limited available literature showing that children with MPNs can develop thrombotic complications. In our cohort, children who developed thromboses were predominantly female and JAK2V617F-mutation-positive. Budd-Chiari syndrome was the most common form of thrombosis. Further study is needed to better understand the pathophysiology and natural history of thromboses in our young patients with MPNs.
To cite this abstract in AMA style:Kucine N, Vanderpoel V, Shimano K. Thrombotic Complications in a Cohort of Children with Myeloproliferative Neoplasms [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/thrombotic-complications-in-a-cohort-of-children-with-myeloproliferative-neoplasms/. Accessed October 19, 2021.
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