Background:The burden of multiple myeloma (MM) is increasing over time in China. However, there is limited data to evaluate comprehensively the epidemiological characteristics of MM.
Methods: Following the general analytical strategy used in GlobalBurden of Disease, Injuries, and Risk Factors Study 2019, we analysed the burden of MM including incidence, mortality, prevalence and disability-adjusted life years (DALYs) with 95% uncertainty interval (UI) in China. Trends in burden of MM from 1990 to 2019 were evaluated.Results: There were an estimated 347.45 thousands DALYs with an age-standardized DALYs rate of 17.05 (95% UI, 12.31-20.77) per 100,000 population in 2019. The estimated incidence case and deaths of MM were 18.79 thousands and 13.42 thousands, with agestandardized incidence and mortality rates of 0.93 (95%UI, 0.67-1.15) and 0.67 (95%UI, 0.50-0.82) per 100 000 population. The agespecific DALYs rates per 100,000 population increased more than 10 at age group 40-44 and reached a peak (93.82) at age group 70-74.Males had higher burden than females with about 1.5-2 folds sexual differences in age-specific DALYs rates in all age groups. From 1990 to 2019, the DALYs number of MM increased by 134% with changing from 148.48 thousands in 1990 to 347.45 thousands in 2019.
Conclusion:The burden of MM increased doubly during the past 3 decades, which highlighted the need of establishment of effective disease prevention and control strategies in both national and provincial levels.
BRAF and NRAS mutations in LCH suggest a possibility of the disease being driven by the activation of the MAPK/ERK pathway. These oncogenic mutations provide new opportunities in understanding LCH pathogenesis and may be a potential target of therapy.Key words: Langerhans cell histiocytosis - mutations - prognostic factors - relapse - survival.
Background:Peripheral T-cell lymphomas (PTCLs) is a diverse group of lymphomas (10-15% of all non-Hodgkin's lymphomas) with aggressive behavior. Despite the standard of 1 st line anthracycline-contain ing regimens, clinical outcomes are poor compared to B-cell lymphomas. In addition, there are still debates about specific prognostic factors (PF) in PTCLs. Aims: Primary endpoints -event-free survival (EFS) and over all survival (OS). To evaluate the prognostic significance of five PTCLs scores (International Prognostic Index -IPI, International Peripheral T-cell lymphoma Project Score -IPTCL, Prognostic Index for T-cell lymphoma -PIT, modified Prognostic Index for T-cell lymphoma -mPIT and T-cell score). Patients and methods: From 67 enrolled patients, only 50 were included: PTCL not otherwise specified (22, 44%), anaplastic large cell lymphoma ALK+ (anaplastic lymphoma kinase-positive) (10, 20%) and ALK− (anaplastic lymphoma kinase-negative) (18, 36%). Patients received CHOP-like regimens (CHOP, CHOEP, EPOCH). Results: The over all rate response was observed in 66% of cases (complete response 78%). There were 48% of relapses after the 1 st line ther apy dur ing follow-up (median 11 months; range 1-85 months). Median age 57 (range 22-80) with male predominance 62%. In total, 40% of patients were > 60 years old, 48% had stage III-IV. Majority of patients were assessed by five prognostic scores. IPI (45 patients): the 3-year EFS and OS were higher for IPI ≤ 1 vs. IPI > 2 (80 vs. 18% and 87 vs. 27%, respectively; p = 0.0002). Receiver operat ing characteristic analysis confirmed poor clinical outcome to patients with PF > 1 (Se = 88 %; Sp = 68 %; AUC = 0.7; p = 0.0081). IPTCLP (41 patients): the presence of PF = 1-2 showed EFS and OS reduction. A 3-year EFS rate for 1-2 PF was 25 vs. 70% for PF = 0 (p = 0.003). Thus, 3-year OS in patients with PF = 0 vs. PF = 1-2 was 100 vs. 20% (p = 0.0001). PIT (42 patients): better 3-year EFS and OS in patients with PF = 0 vs. PF = 1-3 (88 vs. 28% and 100 vs. 34%, respectively, p = 0.001). Patients with PF = 1-3 have a higher rate of relapses vs. PF = 0 (p = 0.0005 by Cox-test). mPIT (21 patients): no significant difference between PF and clinical outcomes. T-cell score (18 patients): higher survival rates with PF ≤ 2. More than 2 PF have an impact on EFS (p = 0.005). The 3-years OS in patients with PF ≤ 2 was 77 vs. 25% in cases with PF ≥ 3 (p = 0.001). Conclusion: IPI, PIT, IPTCLP are still very useful in defin ing risk stratification. As to mPIT and T-cell score, more patients to evaluate their prognostication possibility are needed.
Key wordsperipheral T-cell lymphomas -prognostic factors -peripheral T-cell lymphomas not otherwise specified -anaplastic lymphoma kinase-positive -anaplastic lymphoma kinase-negativeThe authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.Autoři deklarují, že v souvislosti s předmětem studie nemají žádné komerční zájmy.The Editorial Board declares that the manuscript met the ICMJE r...
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