Background: Mantle cell lymphoma (MCL) is a B cell malignancy accounting for 3-10% of all Non-Hodgkin Lymphomas and arises mainly in older adults with a male predominance (McKey et al). Historically, median survival was 4-5 years (Herrmann et al, 2009) however this is now in the order of 8-12 years in younger fitter patients who are able to tolerate intensive therapies (Eskelund et al, 2016). The current standard treatment is chemoimmunotherapy with or without autologous stem cell transplantation (ASCT). Although intensive therapy can achieve durable responses most patients eventually succumb to their disease. Novel therapeutic agents have shown efficacy in relapsed/refractory disease and are now being tested as frontline treatment (Sharma et al, 2018). Aims: We report a single centre experience of MCL and its treatment. Methods: Retrospective data was collected on all patients diagnosed with MCL at Ipswich Hospital NHS Trust over a nine year period between January 2010 and January 2019. Results: Forty-two patients were included in the analysis. Median age was 68yrs with a male to female ratio of 5:1. Eastern Co-operative Oncology Group (ECOG) performance status ranged from 0-1 in 66%, ≥2 in 14% and 19% had no status documented. Twenty-four patients (57%) received a non-intensive chemoimmunotherapy regimen; rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) or rituximab-bendamustine (BR) as first line treatment with twenty of these patients receiving rituximab maintenance. Thirteen patients (31%) were fit enough to receive an intensive chemoimmunotherapy regimen with all but one consolidated with an ASCT. Of those transplanted five went on to receive rituximab maintenance. The remaining 12% of patients received either R-Chlorambucil, palliative radiotherapy, FCR or supportive care. The median Kaplan-Meier overall survival (OS) for all patients had not been reached. Median progression free survival (PFS) was 48 months. Comparing first line therapies, the median OS for those that received RCHOP/BR was 60 months with a median PFS of 35 months vs. not reached for both OS and PFS with intensive chemoimmunotherapy followed by ASCT. Eighteen patients had relapsed during the nine year period. There was no standard second line treatment. Eight patients received further chemoimmunotherapy including BR, R-cytarabine and FCR, one PEP-C, three ibrutinib, four palliative radiotherapy and two received supportive care only. After first relapse median PFS was 6 months and median OS was only 10 months. Nine patients received a third line treatment, of which 3 received ibrutinib and one venetoclax and the median OS was 9 months. The six relapsed/refractory patients that received Ibrutinib at either first or second relapse had a median OS of 8 months vs. 12 months in those that received other forms of therapy. Summary/Conclusion: As expected the best outcomes were with high intensity regimens consolidated by ASCT. Our data period was not long enough to show the continuous pattern of relapse despite durab...

Large granular lymphocytic leukemia (T-LGL) characterized by steady increase in large granular lymphocyte counts over 2*109/l in peripheral blood. T-LGL frequently manifested with splenomegaly, neutropenia, anemia, lymphocytosis, and complicated by infectious diseases. T-LGL cells have mature, post-thymic immune phenotype expressing CD3+,TCRab, CD8+, CD57+ etc. In up to 1/3 of cases T-LGL is combined with autoimmune diseases like rheumatoid arthritis (RA) and could be misdiagnosed asFelty'ssyndrome. Differential diagnosis requiresclonalitytesting. However admixture of polyclonal large granular lymphocytes may compromise PCR sensitivity and decrease the diagnostic value of the test. CD57 + is a typical, but not strictly specific marker of T-LGL cells. Therefore, enrichment in CD57+ cells may circumvent PCR sensitivity issues. This study aimed to assess the possibility and feasibility of clonal assay performed on a selected CD57+ population of peripheral blood lymphocytes in patients with T-LGL for differential diagnosis/verification. The study included 13 patients with RA and assumed T-LGL diagnosis. CD57+ lymphocytes were isolated by means ofimmunomagneticselection onMiltenyiBiotecbeads.T-cellclonalitywas assessed by T-cell receptor gamma chain TCRG (VG - JG) gene rearrangements using multiplex PCR with BIOMED-2 primer system and subsequent Gene-Scan analysis. Polyclonal pattern in bulk lymphocyte population was observed in patients ## 1-3 (Table 1). CD57+ enrichment allowed to determineclonalityand approve T-LGL diagnosis for patient #1 (Figure1). Due to the lack ofclonalityin CD57+ population T-LGL diagnosis was excluded for patient #2. Patient #3 showedoligoclonalpattern in CD57+ enriched population. Bulk lymphocyte population in 5 of 13 patients was monoclonal. After CD57+ enrichment 3 of these 5 patients showed identical monoclonal pattern. Other two patients aquiredadditional peaks (Figure2). In 3 of 13 patients monoclonal peak exceeded the polyclonal background less than 3 times and was interpreted as doubtful monoclonal result. After CD57+ enrichment oligoclonalpattern was detected in these cases. 2 of 13 patients preserved oligoclonalpattern for both bulk and CD57+ enriched lymphocyte populations. CD57+ selection was supportive for diagnostic procedure in 2 cases so far. Based on CD57+ population analysis in one case T-LGL diagnosis was approved while in other excluded. All other included cases gained no benefit from CD57+ enrichment. Frequentoligoclonalpattern in T-LGL may reflect restriction of TCR repertoire of effector cells. We conclude that CD57+ enrichment forclonalitystudies in T-LGL is of limited value for diagnostic procedure in clinical practice. At least at the present level of understanding of T-LGL pathogenesis and reactive lymphocyte populations involved. Table 1 Results of TCRGclonalityassessment in patients with RA and assumed T-LGL diagnosis for bulk and CD57+ enriched lymphocyte population. Table 1. Results of TCRGclonalityassessment in patients with RA and assumed T-LGL diagnosis for bulk and CD57+ enriched lymphocyte population. Figure 1 Results of TCRGclonalityassessment for patient #1 with polyclonal pattern in bulk population (A) and monoclonal pattern in CD57+ enriched lymphocyte population (B). Figure 1. Results of TCRGclonalityassessment for patient #1 with polyclonal pattern in bulk population (A) and monoclonal pattern in CD57+ enriched lymphocyte population (B). Figure 2 Results of TCRGclonalityassessment for patient #7 with monoclonal pattern in bulk population (A) andoligoclonalpattern in CD57+ enriched lymphocyte population (B). Figure 2. Results of TCRGclonalityassessment for patient #7 with monoclonal pattern in bulk population (A) andoligoclonalpattern in CD57+ enriched lymphocyte population (B). Disclosures No relevant conflicts of interest to declare.