红细胞增多症
骨髓纤维化
医学
疾病
真性红细胞增多症
原发性血小板增多症
骨髓增生性肿瘤
人口
骨髓增生性疾病
年轻人
髓样
骨髓
儿科
内科学
肿瘤科
环境卫生
作者
Hannah Goulart,Lucia Masárová,Ruben A. Mesa,Claire Harrison,Jean‐Jacques Kiladjian,Naveen Pemmaraju
摘要
Myeloproliferative neoplasms (MPN) are characterized by a clonal proliferation of myeloid lineage cells within the bone marrow. The classical BCR-ABL negative MPNs are comprised of polycythaemia vera, essential thrombocythaemia and primary myelofibrosis. Historically, the majority of MPNs are diagnosed in adults older than 60 years of age; however, in recent years, there has been recognition of MPNs in the adolescent and young adult (AYA) population. AYAs with MPN, typically defined as between the ages of 15 and 39 years old, may comprise up to 20% of patients diagnosed with MPN. They demonstrate unique patterns of driver mutations and thrombotic events and remain at risk for progression to more aggressive disease states. Given the likely long length of time they will live with their disease, there is a significant unmet need in identifying well-tolerated and effective treatment options for these patients, particularly with the advent of disease modification. In this review, we provide a comprehensive overview of the clinical features, disease course and management of AYA patients with MPN and, in doing so, highlight key characteristics that distinguish them from their older counterparts.
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