BACKGROUND Hepatosplenic T-cell lymphoma(HSTCL)is a rare and aggressive peripheral T-cell lymphoma with historically dismal outcomes,representing less than one percent of non-Hodgkin lymphomas.Given its rarity,the tru...BACKGROUND Hepatosplenic T-cell lymphoma(HSTCL)is a rare and aggressive peripheral T-cell lymphoma with historically dismal outcomes,representing less than one percent of non-Hodgkin lymphomas.Given its rarity,the true incidence of HSTCL is unknown and most data have been extrapolated through case reports.To the best of our knowledge,the largest and most up to date study addressing the epidemiology and outcomes of patients with HSTCL in the United States covered a period from 1996 to 2014,with a sample size of 122 patients.AIM To paint the most updated epidemiological picture of HSTCL.METHODS A total of 186 patients diagnosed with HSTCL,between 2000 and 2017,were ultimately enrolled in our study by retrieving data from the Surveillance,Epidemiology,and End Results database.We analyzed demographics,clinical characteristics,and overall mortality(OM)as well as cancer-specific mortality(CSM)of HSTCL.Variables with a P value<0.01 in the univariate Cox regression were incorporated into the multivariate Cox model to determine the independent prognostic factors,with a hazard ratio of greater than 1 representing adverse prog-nostic factors.CONCLUSION Overall,the outlook for this rare malignancy is very grim.In this retrospective cohort study of the United States population,non-Hispanic blacks and the elderly had a higher CSM.This data highlights the need for larger prospective studies to investigate factors associated with worse prognosis in one ethnic group,such as treatment delays,which have been shown to increase mortality in this racial/ethnic group for other cancers.展开更多
BACKGROUND Extramedullary multiple myeloma(MM)(EMM)is a rare and aggressive subentity of MM that can be present at diagnosis or develop anytime during the disease course.There is a paucity of data on the clinical char...BACKGROUND Extramedullary multiple myeloma(MM)(EMM)is a rare and aggressive subentity of MM that can be present at diagnosis or develop anytime during the disease course.There is a paucity of data on the clinical characteristics and overall epidemiology of EMM.Furthermore,there is a scarcity of data on how the interaction of age and gender influences the survival of EMM.AIM To evaluate the clinical characteristics of patients with EMM over the past 2 decades and to identify epidemiologic characteristics that may impact overall prognosis.METHODS A total of 858 patients diagnosed with EMM,between 2000 and 2017,were ultimately enrolled in our study by retrieving the Surveillance,Epidemiology,and End Results database.We analyzed demographics,clinical characteristics,and overall mortality(OM)as well as cancer-specific mortality(CSM)of EMM.Variables with a P value<0.1 in the univariate Cox regression were incorporated into the multivariate Cox model to determine the independent prognostic factors,with a hazard ratio(HR)of greater than 1 representing adverse prognostic factors.RESULTS From a sample of 858 EMM,the male gender(63.25%),age range 60-79 years(51.05%),and non-Hispanic whites(66.78%)were the most represented.Central Nervous System and the vertebral column was the most affected site(33.10%).Crude analysis revealed higher OM in the age group 80+[HR=6.951,95%confidence interval(95%CI):3.299-14.647,P=0],Non-Hispanic Black population(HR=1.339,95%CI:1.02-1.759,P=0.036),Bones not otherwise specified(NOS)(HR=1.74,95%CI:1.043-2.902,P=0.034),and widowed individuals(HR=2.107,95%CI:1.511-2.938,P=0).Skin involvement(HR=0.241,95%CI:0.06-0.974,P=0.046)and a yearly income of$75000+(HR=0.259,95%CI:0.125-0.538,P=0)had the lowest OM in the crude analysis.Crude analysis revealed higher CSM in the age group 80+,Non-Hispanic Black,Bones NOS,and widowed.Multivariate cox proportional hazard regression analyses only revealed higher OM in the age group 80+(HR=9.792,95%CI:4.403-21.774,P=0)and widowed individuals(HR=1.609,95%CI:1.101-2.35,P=0.014).Multivariate cox proportional hazard regression analyses of CSM also revealed higher mortality of the same groups.Eyes,mouth,and ENT involvement had the lowest CSM in the multivariate analysis.There was no interaction between age and gender in the adjusted analysis for OM and CSM.CONCLUSION EMM is a rare entity.To our knowledge,there is a scarcity of data on the clinical characteristics and prognosis factors of patients with extramedullary multiple myeloma.In this retrospective cohort,using a United States-based population,we found that age,marital status,and tumor site were independent prognostic factors.Furthermore,we found that age and gender did not interact to influence the mortality of patients with EMM.展开更多
文摘BACKGROUND Hepatosplenic T-cell lymphoma(HSTCL)is a rare and aggressive peripheral T-cell lymphoma with historically dismal outcomes,representing less than one percent of non-Hodgkin lymphomas.Given its rarity,the true incidence of HSTCL is unknown and most data have been extrapolated through case reports.To the best of our knowledge,the largest and most up to date study addressing the epidemiology and outcomes of patients with HSTCL in the United States covered a period from 1996 to 2014,with a sample size of 122 patients.AIM To paint the most updated epidemiological picture of HSTCL.METHODS A total of 186 patients diagnosed with HSTCL,between 2000 and 2017,were ultimately enrolled in our study by retrieving data from the Surveillance,Epidemiology,and End Results database.We analyzed demographics,clinical characteristics,and overall mortality(OM)as well as cancer-specific mortality(CSM)of HSTCL.Variables with a P value<0.01 in the univariate Cox regression were incorporated into the multivariate Cox model to determine the independent prognostic factors,with a hazard ratio of greater than 1 representing adverse prog-nostic factors.CONCLUSION Overall,the outlook for this rare malignancy is very grim.In this retrospective cohort study of the United States population,non-Hispanic blacks and the elderly had a higher CSM.This data highlights the need for larger prospective studies to investigate factors associated with worse prognosis in one ethnic group,such as treatment delays,which have been shown to increase mortality in this racial/ethnic group for other cancers.
文摘BACKGROUND Extramedullary multiple myeloma(MM)(EMM)is a rare and aggressive subentity of MM that can be present at diagnosis or develop anytime during the disease course.There is a paucity of data on the clinical characteristics and overall epidemiology of EMM.Furthermore,there is a scarcity of data on how the interaction of age and gender influences the survival of EMM.AIM To evaluate the clinical characteristics of patients with EMM over the past 2 decades and to identify epidemiologic characteristics that may impact overall prognosis.METHODS A total of 858 patients diagnosed with EMM,between 2000 and 2017,were ultimately enrolled in our study by retrieving the Surveillance,Epidemiology,and End Results database.We analyzed demographics,clinical characteristics,and overall mortality(OM)as well as cancer-specific mortality(CSM)of EMM.Variables with a P value<0.1 in the univariate Cox regression were incorporated into the multivariate Cox model to determine the independent prognostic factors,with a hazard ratio(HR)of greater than 1 representing adverse prognostic factors.RESULTS From a sample of 858 EMM,the male gender(63.25%),age range 60-79 years(51.05%),and non-Hispanic whites(66.78%)were the most represented.Central Nervous System and the vertebral column was the most affected site(33.10%).Crude analysis revealed higher OM in the age group 80+[HR=6.951,95%confidence interval(95%CI):3.299-14.647,P=0],Non-Hispanic Black population(HR=1.339,95%CI:1.02-1.759,P=0.036),Bones not otherwise specified(NOS)(HR=1.74,95%CI:1.043-2.902,P=0.034),and widowed individuals(HR=2.107,95%CI:1.511-2.938,P=0).Skin involvement(HR=0.241,95%CI:0.06-0.974,P=0.046)and a yearly income of$75000+(HR=0.259,95%CI:0.125-0.538,P=0)had the lowest OM in the crude analysis.Crude analysis revealed higher CSM in the age group 80+,Non-Hispanic Black,Bones NOS,and widowed.Multivariate cox proportional hazard regression analyses only revealed higher OM in the age group 80+(HR=9.792,95%CI:4.403-21.774,P=0)and widowed individuals(HR=1.609,95%CI:1.101-2.35,P=0.014).Multivariate cox proportional hazard regression analyses of CSM also revealed higher mortality of the same groups.Eyes,mouth,and ENT involvement had the lowest CSM in the multivariate analysis.There was no interaction between age and gender in the adjusted analysis for OM and CSM.CONCLUSION EMM is a rare entity.To our knowledge,there is a scarcity of data on the clinical characteristics and prognosis factors of patients with extramedullary multiple myeloma.In this retrospective cohort,using a United States-based population,we found that age,marital status,and tumor site were independent prognostic factors.Furthermore,we found that age and gender did not interact to influence the mortality of patients with EMM.
