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Identifying Bone Marrow Microenvironmental Populations in Myelodysplastic Syndrome and Acute Myeloid Leukemia
Published on: November 10, 2023
[Clinical features and prognostic factors in primary myelofibrosis patients under 45 years old]
Lin Lu1, Zhi-jian Xiao, Hui-shu Chen
1Institute of Hematology and Blood Disease Hospital, CAMS & PUMC, Tianjin 300020, China.
Insights
Young primary myelofibrosis (PMF) patients with low hemoglobin and constitutional symptoms face a poor prognosis. These factors identify high-risk individuals with survival potentially less than three years.
Area of Science:
- Hematology
- Oncology
- Internal Medicine
Context:
- Primary myelofibrosis (PMF) is a rare bone marrow cancer.
- Young PMF patients (<45 years) represent a distinct clinical subgroup.
- Identifying prognostic factors is crucial for risk stratification and management.
Purpose:
- To investigate clinical and hematological features of young PMF patients.
- To identify prognostic factors for short-term survival in this cohort.
- To stratify risk based on identified prognostic factors.
Summary:
- This study analyzed 56 primary myelofibrosis patients under 45 years old.
- Low hemoglobin (<100 g/L) and constitutional symptoms were independently associated with short survival.
- These factors effectively stratified patients into high-risk (median survival 16 months) and low-risk (median survival 88 months) groups.
Impact:
- Establishes key prognostic markers for young PMF patients.
- Enables improved risk stratification and personalized treatment strategies.
- Highlights the critical role of hemoglobin levels and constitutional symptoms in predicting outcomes.
Objective:
To investigate the clinical and hematological features and identify the prognostic factors associated with short-term survival in primary myelofibrosis (PMF) patients under 45 years old.
Methods:
The clinical manifestations, laboratory parameters, and survival were retrospectively analyzed in 56 PMF patients under 45 years old. Univariate analysis of prognostic factors was performed using Logrank test and multivariate analysis using COX model.
Results:
Of the 56 patients, 27 were males and 29 females. The range of age was from 20 to 45 years (median 38 years). 84% of the patients were anemic and 66% Hb < 100 g/L. 32% of the patients had constitutional symptoms including fever, night sweats and weight loss. The median survival was 69 months (95% CI 11-127). By univariate analysis, Hb < 100 g/L, platelet count < 100 x 10(9)/L, WBC < 10 x 10(9)/L, constitutional symptoms and the duration from first signs to diagnosis < or = 6 months were associated with poor prognosis. By multivariate analysis, only Hb < 100 g/L and constitutional symptoms were independently associated with short survival. With these two adverse prognostic factors, the patients could be separated into a high risk and a low risk groups, and the median survivals were 16 and 88 months, respectively (P < 0.001). Using these two factors to predict the less than 3-years survival for individual patient, the sensitivity, specificity and positive predictive value were 67%, 100% and 100%, respectively.
Conclusion:
Hb < 100 g/L and constitutional symptoms in PMF patients under 45 years old were significantly associated with short survival and poor prognosis. These two prognostic factors enabled to separate patients into a high and a low risk groups. The survival of high-risk patients was less than three years.
