PB2182 OUTCOME OF FIRST‐LINE THERAPY FOR PATIENTS WITH SYSTEMIC LIGHT‐CHAIN AMYLOIDOSIS: MONOCENTRIC ANALYSIS

Background:Systemic light‐chain amyloidosis (AL) is characterised by deposition of misfolded immunoglobulin light chains within organs.Melphalan or cyclophosphamide with dexamethasone in combination with bortezomib are currently recommended first‐line therapy regimens.Aims:To describe the clinical presentation and outcomes for newly diagnosed AL amyloidosis patients.MethodsThis single‐centre retrospective study included all patients diagnosed with AL amyloidosis in our hospital area (300,000 inhabitants) between March 2012 to March 2018. Data regarding demography, diagnosis, treatment and follow‐up were extracted from the clinical history (HC).Survival curves were constructed according to the Kaplan‐Meier method and compared by logarithmic ranking.Time to progression (TTP) was defined as the time from the date of diagnosis or the start of a treatment for a disease until the disease worsens.Results:We analysed 24 patients (15women, 9 men) with newly diagnosed AL amyloidosis. Median age was 66 years (range 42‐82), and 6 pts were considered ASCT candidates. Involvement of kidney, peripheral nerve, heart, liver and other organs were found in 14 (35%), 13 (32.5%), 11 (24.5%), 1 (2.5%) and 1 (2.5%) cases, respectively.According to Mayo Stage 2012: stage 2: 25%, stage3: 33.33% and stage 4: 41.66%First‐line treatments were: regimens based on bortezomib CyBordex (cyclophosphamide, bortezomib and dexamethasone) n = 12, VTD (bortezomib, thalidomide and dexamethasone) n = 2, VMP (bortezomib, melphalan and prednisone) n = 2, BD (bortezomib and dexamethasone) n = 6, MP (melphalan and prednisone) n = 2 and CP (cyclophosphamide and prednisone) n = 1.Haematological responses of CyBordex, VTD, VMP, BD were 33.33%, 100% and 100% and 16.66% respectively.The haematological responses were: RC 25%, VGPR 33.33%, RP 8.33%, NR (no response) 33.33%The organ responses were 45.83% (n = 11). Median follow‐up of 19 months (95% CI 0‐49.9). Median PTT of 19 months (95% CI 0‐41.9).The rate of premature death within the first six months was 29.16%.Summary/Conclusion:Regimens based on bortezomib in newly diagnosed AL amyloidosis show results that seem comparable to those previously published in the literature.Almost a third of patients show no response, and premature death in six months is significative.To improve these outcomes, it is necessary to better define the role of proteasome inhibitors the role of new therapies.Educational interventions are important to optimize the diagnostic process and to reduce the rate of early deaths.