Primary plasma cell leukemia (pPCL) is an uncommon but aggressive plasma cell malignancy associated with frequent extramedullary involvement, high-risk cytogenetic abnormalities, and frequent organ dysfunction, ultimately resulting in poor prognosis. Here we review recent advances in our understanding of the molecular and biological aspects of PCL and summarize therapeutic progress occurring over the past 2 decades. pPCL is distinguished from secondary PCL arising from multiple myeloma. The molecular and immunophenotypic changes of pPCL are often distinct from those seen in secondary PCL and multiple myeloma. The availability of novel agents (ie, proteasome inhibitors and immunomodulatory agents) and the increasing use of hematopoietic cell transplantation strategies have resulted in better outcomes, although long-term survival remains poor. Development of complex treatment algorithms that combine novel agents as induction therapy, as part of conditioning regimens for hematopoietic cell transplantation (autologous or allogeneic), or as post-transplantation remission strategies are logical and may translate into improved survival in patients with PCL.
e12525 Background: The objective of this study was to determine the clinicopathologic characteristics of Lebanese patients with breast cancer below 40 compared to those above 40 presenting to our institution. Methods: Using a cross-sectional study design, data were collected prospectively on a sample of 110 women newly diagnosed with breast cancer. Comparison between the groups was performed using Person Chi-square and Fishers exact test. Results: 28 patients were ≤ 40 years and 82 patients >40 years. 75% and 76.5% of patients above and below 40 years respectively presented with stage I or II disease, more patients above 40 presented with stage I compared to those below 40 (32.4% vs 8% respectively). 50% of the younger age group presented with a grade 3 tumor; however, more than the half of the older group presented with grade 1 or 2. The older age group had a higher incidence of concurrent lobular and intraductal carcinoma (0 vs 6.4%). The younger age group was more likely to present with nodal involvement (57.1% vs. 48.45 for younger and older age groups respectively). Similar proportions of the two age groups stained positive for cyclin B1, p53, and VEGFR. All patients stained positive for PDGFR. Mean value of Ki-67 was 21.8% and 24%in younger and older age groups respectively. These differences were not statistically significant. Methylene Tetrahydrofolate Reductase (MTHFR) single nuccleotide polymorphism (SNP) T677T was detected in 21% of the older group compared to 5% of the younger population. Stratified to age Vitamin D receptor (VDR) SNP ApaI A/A was found in similar proportion in both groups (35% and 33.3% in younger and older group) while 30% and 41.3 % carried the vitamin D receptor (SNP) BSMI b/b in younger and older group respectively. Conclusions: No significant differences in the clinicopathologic characteristics examined between patients younger or older than 40 years were found in this sample. Analysis of a larger sample size including BRCA gene mutation analysis is planned to determine differences between age groups and possibly explain the higher incidence of breast cancer in the younger age group in our population.
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