Plasma cell leukemia (PCL) is a rare and aggressive plasma cell disorder, with poor outcome. Bortezomibbased regimens (BBR) are highly effective in myeloma, but there is limited information about their efficacy and safety in PCL. Thus, we retrospectively collected data from 42 consecutive PCL patients (25 with primary PCL-pPCL and 17 with secondary PCL-sPCL) to explore the role of BBR in this entity. BBR were administered in 29 of 42 patients, while 6 of 25 patients with pPCL underwent autologous transplantation. Objective response (partial response) was significantly higher in patients treated with BBR versus conventional therapies (69% vs. 30.8%, P 5 0.04); 27.5% of patients treated with BBR achieved at least very good partial response (vgPR). The highest ORR was observed in pPCL patients treated with BBR (88.9%; vgPR: 33.3%). In BBR-group, grade 3 of 4 hematological, neurological and renal toxicity and neutropenic infections were observed in 41.4%, 7%, 3.4%, and 31%, respectively. With a median follow-up of 51 months, median overall survival (OS) for patients treated with BBR versus conventional therapies was 13 versus 2 months (P < 0.007). Median OS of patients with pPCL and sPCL treated with BBR was 18 and 7 months, respectively (P < 0.001). In the multivariate analysis normal PLTs, treatment with BBR and high quality response were the only powerful predictors for survival. Our study carrying the longest reported median follow-up, demonstrated that treatment of PCL with BBR induces high response rates and prolongs survival over conventional therapies, regardless of additional autologous transplantation rescue or established high risk features, with manageable toxicity.

Solitary plasmacytoma (SP) is a rare plasma cell dyscrasia characterized by the presence of bone or extramedullary plasma cell tumors. The treatment of choice is local radiotherapy (R/T) 6 surgical excision. The role of adjuvant chemotherapy (C/T) or novel agents (NA) is uncertain. Data related to prognostic factors are inconclusive. Herein, we describe the clinical features, survival and prognosis of 97 consecutive patients, 65 with bone SP (SBP), and 32 with extramedullary SP (SEP), diagnosed and treated in 12 Greek Myeloma Centers. Objective response rate (PR) and complete response (CR) was 91.8% and 61.9%, respectively, and did not differ between the 2 groups. Overall, 38 patients relapsed or progressed to multiple myeloma (MM). After a median follow-up of 60 months, 5 and 10-year overall survival (OS) probability was 92% and 89% in SEP and 86% and 69% in SBP, respectively (P 5 0.2). The 5-and 10-year MMfree survival (MMFS) probability was 90% and 70% for patients with SEP vs. 59% and 50% for patients with SBP, respectively (P 5 0.054). Overall, the 5-and 10-year OS probability, plasmacytoma relapse-free survival (PRFS), progression-free survival and MMFS was 84% and 78%, 72% and 58%, 58% and 43%, and 70% and 59%, respectively. In the multivariate analysis, prolonged PRFS and young age were positive predictors of OS. Achievement of CR was the only positive predictor of PRFS. Immunoparesis was the only negative predictor of progression to MM. The addition of C/T or NA-based treatment increased toxicity without offering any survival advantage over R/T.

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The impact of erythropoiesis-stimulating agent (ESA) on cancer patients' survival has recently become a matter of extensive discussion. Studies in solid tumors demonstrated that ESA adversely affects survival. This issue has not been sufficiently studied in patients with multiple myeloma. In this study, which included 323 multiple myeloma patients followed in our Institution between 1988 and 2007, we demonstrated by using a proportional hazards model including multiple covariates (age, LDH, Hb, platelets, serum creatinine, ISS score, b2 microglobulin, and ESA administration) that ESA administration is associated with reduced survival (hazards ratio: 1.88, 95% CI: 1.28-2.77). Anemia, which is considered a predictor for survival, platelets, serum creatinine, ISS score, and LDH, were not significant, whereas, age and b2 microglobulin confirmed their predicting value in the multivariate analysis. With a median follow-up of 31 months (range 1-238), the median survival of patients in the ESA group was 31 months (95% CI: 25-37), whereas in the group without ESA administration it was 67 months (95% CI: 55-79) (P < 0.001). The median progressionfree survival for patients in the ESA group was 14 months (95% CI: 12-16), and for the group without ESA it was 30 months (95% CI: 24-36) (P < 0.001). These results indicate that ESA may have a detrimental impact on MM patients' outcomes and, thus, in this context, they should be used with rigorous criteria. Am. J. Hematol. 83:697-701, 2008. V

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Solitary plasmacytoma (SP) is a rare plasma cell dyscrasia characterized by the presence of bone or extramedullary tumors consisting of monoclonal plasma cells, without evidence of systematic multiple myeloma (MM). The treatment of choice is local radiotherapy (RT) with or without surgical excision (SE). Our aim was to describe the clinical features, the outcome and prognosis of SP patients. Thus, we studied 97 consecutive patients, 65 with solitary bone plasmacytoma (SBP) and 32 with solitary extramedullary plasmacytoma (SEP), (M/F: 66/31, median age: 61 years, range 17-85 years) who were diagnosed and treated in 12 Greek Myeloma Centers; treatment was given according to each center’s local policy. Patients with SBP and SEP did not differ in age, gender, performance status (PS), Hb, CRP, LDH, calcium, beta2-microglobulin, renal function or impairment of humoral immunity (immunoparesis). Patients with SEP presented more often with serum or urinary monoclonal component (p<0.001 and p=0.004, respectively). The median size of SP was larger in patients with SBP compared to patients with SEP (5.5cm vs. 3cm; p=0.003). CD56 antigen was expressed more often on plasma cells of SBP biopsies (p=0.03). Eighteen (56.2%) patients with SEP presented with upper respiratory track plasmacytoma and 14 presented with plasmacytomas in other locations. In 26/65 patients (40%) with SBP, plasmacytoma was located in the vertebrae and 39 had plasmacytoma in other bones. Bisphosphonates were administered more frequently in SBP patients (p=0.001). There was no difference in the type of treatment (RT, conventional chemotherapy (CT) ± novel agents (NA), SE or combined treatment) between the 2 groups; 80 patients (82.5%) received RT alone or in combination with CT and/or SE, 7 patients (7.2%) received CT alone, 7 patients (7.2%) underwent only SE and 3 patients (3.1%) were treated with CT and SE. The median dose of radiation delivered was 40 Gy (range 24-55 Gy). Novel agent combinations were administered in 27/47 patients (57.4%) who received CT (bortezomib-based regimens: 46.8%); 13/47 (27.6%) patients treated with CT underwent high-dose therapy. Myelotoxicity, neurotoxicity and neutropenic infections presented more frequently in patients treated with CT (p<0.001, p=0.01, p=0.009, respectively). Objective response rate (≥PR) and complete response (CR) was 91.8% and 61.9%, respectively. There was no difference in response rates or median time to response (3 months, range 1-24 months) between patients with SBP or SEP. Overall, 38 (39.2%) patients relapsed (relapse of SP only: 14; progression to MM only: 16, relapse of SP and progression to MM: 8). After a median follow up of 60 months (range 3-264 months), 74 (76.3%) patients were alive, 13 (13.4%) were dead and 10 (10.3%) patients were lost to follow up. Overall survival (OS), although higher in SEP over SBP, did not reach statistical significance. The 10-year MM-free survival (MMFS) was 50% vs. 70% respectively, for patients with SBP vs. SEP (p=0.054). Ten-year OS for patients treated with RT± surgical excision was 81% vs. 70% for those treated with other therapies including CT± NA (p=0.3). Overall, the 10-year OS, plasmacytoma relapse-free survival (PRFS), progression-free survival (PFS) and MMFS was 78%, 58%, 43% and 59%, respectively. In the multivariate analysis PRFS and age predicted for OS (p=0.02, HR: 0.97; 95% CI: 0.94-0.99 and p=0.01, HR: 1.09; 95% CI: 1.017-1.16, respectively); 10-year OS for patients <60 vs. ≥60 years was 85% vs. 60% (p=0.02); patients with PRFS >24 vs. ≤24 months had a 10-year OS of 82% vs. 60% (p=0.01). Achievement of CR predicted positively for PRFS. Immunoparesis (n=24) predicted negatively for progression to MM. Chemotherapy including NA had no prognostic impact on OS, PRFS or MMFS. Our study demonstrated that patients with SP enjoy a prolonged OS that is favorably influenced by young age and PRFS. We also confirmed that patients with SBP progress more frequently to MM compared to SEP in the era of novel agents. Radiotherapy with or without SE remains the treatment of choice for SP. The additional use of systematic therapy including novel anti-myeloma agents increases toxicity without improving survival or decreasing the probability of SP relapse or progression to MM. Immunoparesis predicts for progression to MM. Disclosures: No relevant conflicts of interest to declare.