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Adolescent and Young Adult versus Older Adult Philadelphia-Negative Myeloproliferative Neoplasms: A Single-Center
Shehab F Mohamed1, Awni Alshurafa1, Dina Sameh Soliman2,3
1Hematology Department, National Center for Cancer Care and Research (NCCCR), Hamad Medical Corporation, Doha, Qatar.
Background:
Philadelphia-negative myeloproliferative neoplasms (MPNs), including essential thrombocythaemia (ET), polycythaemia vera (PV), and myelofibrosis (MF), usually affect older adults, but a subset occurs in adolescents and young adults (AYA). Regional age-stratified data remain limited.
Methods:
We retrospectively studied Philadelphia-negative MPN patients managed at the National Center for Cancer Care and Research from 2014 to 2024. Patients were classified as AYA (15-39 years) or older adults (≥40 years). Clinical, laboratory, molecular, thrombotic, bleeding, transformation, and survival outcomes were analyzed by subtype. Risk categories followed institutional disease-specific practice; analyses were descriptive and unadjusted.
Results:
The cohort included 220 patients: 79 ET, 47 MF, and 94 PV; 76 (34.5%) were AYA. Low-risk disease predominated in AYA ET (p<0.001) and PV (72.4% vs 35.4%; p=0.002), partly reflecting age-based risk definitions. In ET (molecular n=30 vs 49), CALR was 16/30 (53.3%) versus 19/49 (38.8%) and JAK2 was 6/30 (20.0%) versus 21/49 (42.9%); MPL, triple-negative, and unavailable categories were 2, 4, and 2 versus 2, 6, and 1, respectively. In MF (n=11 vs 36), JAK2 was 4 (36.4%) versus 18 (50.0%), CALR 4 (36.4%) versus 10 (27.8%), MPL 0 versus 2 (5.6%), triple-negative 1 (9.1%) versus 3 (8.3%), and unavailable data 2 (18.2%) versus 3 (8.3%). In PV (n=29 vs 65), JAK2 V617F was 28 (96.6%) versus 63 (96.9%) and JAK2 exon 12 was 1 (3.4%) versus 2 (3.1%). Overall survival did not differ by age.
Conclusion:
AYA patients with Philadelphia-negative MPNs demonstrated age-related differences in risk classification, molecular profile, and thrombotic phenotype, while overall survival was comparable to older adults. Larger prospective multicenter studies are warranted.
Insights
Adolescents and young adults (AYA) with Philadelphia-negative myeloproliferative neoplasms (MPNs) show distinct risk classifications and molecular profiles compared to older adults. However, overall survival rates were similar between these age groups, indicating comparable outcomes despite differing disease characteristics.
Area of Science:
- Hematology
- Oncology
- Genetics
Background:
- Philadelphia-negative myeloproliferative neoplasms (MPNs), including essential thrombocythaemia (ET), polycythaemia vera (PV), and myelofibrosis (MF), typically affect older adults.
- A notable subset of MPNs occurs in adolescents and young adults (AYA), but age-stratified data remain limited.
- Understanding age-related differences in MPN presentation and outcomes is crucial for tailored patient management.
Purpose of the Study:
- To investigate and compare clinical, laboratory, molecular, and outcome differences in Philadelphia-negative MPN patients stratified by age (AYA vs. older adults).
- To analyze thrombotic and bleeding events, disease transformation, and survival rates between AYA and older adult MPN cohorts.
- To provide regional age-stratified data on MPN subtypes, addressing a gap in current medical literature.
Main Methods:
- Retrospective analysis of 220 Philadelphia-negative MPN patients managed between 2014 and 2024.
- Patients were categorized as AYA (15-39 years) or older adults (≥40 years).
- Outcomes including clinical presentation, laboratory findings, molecular mutations (JAK2, CALR, MPL), thrombosis, bleeding, transformation, and survival were analyzed by MPN subtype and age group.
Main Results:
- The cohort comprised 76 AYA (34.5%) and 144 older adults across ET, PV, and MF subtypes.
- AYA patients exhibited a higher proportion of low-risk disease in ET and PV, partly due to age-based risk stratification.
- Significant differences in molecular profiles were observed, particularly in ET (CALR and JAK2 mutations) and MF (JAK2 and CALR mutations), while PV showed a consistent JAK2 V617F mutation prevalence across age groups. Overall survival did not differ significantly between AYA and older adult patients.
Conclusions:
- Adolescents and young adults with Philadelphia-negative MPNs present with distinct age-related differences in risk classification, molecular landscape, and thrombotic phenotypes compared to older adults.
- Despite these differences, overall survival remains comparable between AYA and older adult MPN patients.
- Further large-scale, prospective, multicenter studies are essential to validate these findings and refine management strategies for AYA MPN patients.