ABSTRACT
Myeloproliferative neoplasms (MPNs) are a group of chronic hematologic malignancies that lead to morbidity and early mortality due to thrombotic complications and progression to acute leukemia. Clinical and mutational risk factors have been demonstrated to predict outcomes in patients with MPNs and are used commonly to guide therapeutic decisions, including allogenic stem cell transplant, in myelofibrosis. Adolescents and young adults (AYA, age ≤45 years) comprise less than 10% of all MPN patients and have unique clinical and therapeutic considerations. The prevalence and clinical impact of somatic mutations implicated in myeloid disease has not been extensively examined in this population. We conducted a retrospective review of patients evaluated at eight Canadian centers for MPN patients diagnosed at ≤45 years of age. In total, 609 patients were included in the study, with median overall survival of 36.8 years. Diagnosis of prefibrotic or overt PMF is associated with the lowest OS and highest risk of AP/BP transformation. Thrombotic complications (24%), including splanchnic circulation thrombosis (9%), were frequent in the cohort. Mutations in addition to those in JAK2/MPL/CALR are uncommon in the initial disease phase in our AYA population (12%); but our data indicate they may be predictive of transformation to post-ET/PV myelofibrosis.
Subject(s)
Myeloproliferative Disorders , Polycythemia Vera , Primary Myelofibrosis , Thrombocythemia, Essential , Thrombosis , Humans , Young Adult , Adolescent , Middle Aged , Primary Myelofibrosis/genetics , Primary Myelofibrosis/therapy , Polycythemia Vera/genetics , Thrombocythemia, Essential/genetics , Canada/epidemiology , Myeloproliferative Disorders/complications , Myeloproliferative Disorders/genetics , Myeloproliferative Disorders/therapy , Thrombosis/genetics , Janus Kinase 2/genetics , Mutation , Calreticulin/geneticsABSTRACT
The majority of adult mental health and substance use (MH&SU) conditions emerge in adolescence. Prevention, diagnosis, and treatment programs targeting this age group have a unique opportunity to significantly impact the well-being of the future generation of adults. At the same time, youth are reluctant to seek treatment and have high rates of dropout from interventions. An emphasis on youth engagement in prevention and treatment interventions for MH&SU results in better health outcomes for those youth. This literature review was undertaken to evaluate opportunities to improve youth engagement in MH&SU programs. The intent was to determine best practices in the field that combined community-level improvement in clinical outcomes with proven strategies in engagement enhancement to inform program development at a local level. The results discuss 40 studies, reviews, and program reports demonstrating effective youth engagement. These have been grouped into six themes based on the underlying engagement mechanism: youth participation in program development, parental relationships, technology, the health clinic, school, and social marketing. A broad range of tools are discussed that intervention developers can leverage to improve youth engagement in prevention or treatment programs.
