A retrospective analysis was done using the Surveillance, Epidemiology, and End-Results (SEER) database to determine the trends in overall survival and identify prognostic factors in patients with mantle cell lymphoma (MCL). In total 5367 cases of MCL identified from 1992 to 2007 were split into three cohorts, group 1(1992-1999), group 2 (2000-2003) and group 3 (2004-2007). Survival was analyzed using the Cox proportional hazards model to correct for age, gender and stage of disease. The proportion of patients with advanced disease at diagnosis, male gender and advanced age increased over time and these were all associated with increased mortality. The overall survival remained unchanged. However, when adjusted for the increased proportion of patients with poor prognostic features noted above, we found a significant improvement in survival. The adjusted model also showed an improvement in predicted survival over time in patients with advanced stage. No change in survival was seen in patients with localized disease. Although this analysis is not designed to evaluate specific treatment modalities, these data suggest that the development of new treatment strategies over the past decade may be impacting the survival of patients with advanced MCL despite the finding that the overall survival remains unchanged in the general US population.
to the plug formation. This inflammation then leads to fibrosis and atrophy. 4 It may be that TGF-β signaling may also be involved because it is known that TGF-β signaling plays an important role in wound healing and scar formation. In LDS, atrophic scars and delayed wound healing have been reported. 5 Interestingly, TGFBR1 null mutations cause multiple self-healing squamous epithelioma (MSSE) 6 with subsequent atrophic scarring, which is also typical for AV. However, patients with MSSE do not have features of LDS, which is usually caused by missense mutations in the TGFBR1-or TGFBR2-kinase domain. 5 Further investigations are needed to clarify the relationship between AV and LDS. In conclusion, it appears that AV should be added as a cutaneous feature of LDS, possibly more specifically TGFBR2related LDS. In view of the crucial importance of early detection and management of aortopathy in LDS, we suggest a careful evaluation for clinical features of LDS, including cardiac ultrasonography and possibly TGFBR1/2 DNA analysis in every patient presenting with AV.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.