Page 216 - 2020_01-Haematologica-web

M. Beksac et al.
ethasone, different groups have reported conflicting results.27
In a retrospective study, a subset of 101 EMD patients (66 at diagnosis and 35 at relapse), were compared to patients without any EMD but enrolled in Total Therapy (TT) or non-TT protocols.28 Regardless of therapy, EMD was asso- ciated with shorter PFS and OS: EMP at diagnosis was asso- ciated with poor PFS (TT: 27%, non-TT: 12% after 5 years) and OS (TT: 35%, non-TT: 34% after 5 years) regardless of whether or not the patients were treated on TT protocols.14 The PFS and OS in our study is comparable to the survival durations reported by the Arkansas group. Usmani et al., but not Pour et al., found fluorescence in situ hybridization (FISH) detectable abnormalities to be associated with EMD and poor outcome.21,24 In our study, FISH analysis was avail- able in half of the patients and was similar to the experience of the Czech group revealing results comparable to the gen- eral myeloma population. In our experience, only 13q del (18%), was observed less frequently than expected.
In a recent paper, Kumar et al. have analyzed data of 44 (16.2%) EMD out of 271 consecutive ASCT recipients. Although they did not discriminate EMP from PO, both
OS and PFS was shorter for patients with EMD; median OS was 19.2 months (95%CI: 10.6-27.8) with a median PFS of 19 months (95%CI: 12.6-25.4). Achievement of CR post transplant was found to be the most important pre- dictor for OS and PFS in this study.29 In our cohort, 67 myeloma patients with EMD at diagnosis underwent ASCT within a median of 10.7 months and 39 patients (66.1%) achieved ≥very good partial response (VGPR) fol- lowing ASCT. We were also able to demonstrate the impact of transplant on OS in our newly diagnosed EMD ASCT cohort in univariate and multivariate analysis. Although PFS was comparable to the standard myeloma population, we were not able to see the impact of response ≥VGPR, which may be attributable to the differ- ences among the imaging tools used.
The European Group of Blood and Marrow Transplantation (EBMT) recently reported on 682 EMD subjects (EMP/PO: 139/543) who have received ASCT. In this report, PO (14.5%) involvement was found to be more frequent compared to EMP (3.7%). They noted a gradual increase in frequency of EMD from 2005 to 2014.30 Similar to our results, they also report ISS to have a poor
AB
CD
Figure 2. Overall survival (OS) estimates comparing the risk factors in (A) extramedullary disease (EMD) patients at diagnosis according to International Staging System (ISS) stage, (B) EMD patients according to disease stage, (C) all patients according to paraosseous (PO) versus extramedullary plasmacytomas (EMP) and (D) all patients according to autologous stem cell transplantation (ASCT) treatment at diagnosis versus at relapse versus no ASCT.
206
haematologica | 2020; 105(1)