Real World Data on Demographics, Management and Outcome of Ultra High-Risk Myeloma - Experience from a Tertiary Care Centre
摘要
Multiple Myeloma (MM) is a heterogeneous disease and genetic profile plays a major role in prognosis and treatment outcome. Prognostic systems are continuously evolving as treatment is changing and new data on prognostic factors is emerging. Ultra High-Risk Myeloma includes Double-hit (DH) MM (coexistence of two high-risk abnormalities) and triple-hit (TH) MM (coexistence of three high-risk abnormalities). Ultra High-Risk subset is associated with poor prognosis and real-world data on management are limited. Hospital registry of newly diagnosed Multiple Myeloma patients registered between January 2016 and December 2019 were searched to identify patients with ultra-high-risk Myeloma. We retrospectively analyzed the demographics, management and outcome of ultra-high-risk patients. SPSS descriptive statistics were performed for demographic characteristics. Kaplan-Meier plots were used for survival analysis. Univariate analysis was done to see the impact of baseline characteristics on Progression free survival (PFS) and Overall Survival (OS). Between 1st January 2016 and 31st December 2019, 1011 patients were registered as newly diagnosed MM. Of these, 48 (4.75%) were identified to have DH/TH disease. The median age of the population was 54.5 years (Range 28–72 years). Thirty-one patients (64.6%) were Male. ECOG PS was 0–2 in 35 patients (72.9%). Thirty-two patients (66.7%) had ISS III and R-ISS III disease. Forty-three (89.6%) patients had DH and rest five (10.4%) patients had TH Myeloma. Twenty-three (47.9%) patients received PI-based triplet and 22 (45.8%) received PI+IMiD based triplet as initial therapy. Thirty-seven patients (77.1%) attained at least a very good partial response (VGPR) or better as best response with induction chemotherapy. The median PFS was 20 months (95% CI: 0–42.63) and median OS was 25 months (95% CI: 9.48–40.52). Presence of any specific cytogenetic abnormality except t(14;16), did not affect survival outcomes. Similarly, presence of double-hit versus triple-hit did not affect the PFS or OS of the patients. Only five patients underwent auto-HSCT post induction chemotherapy. Patients who received maintenance had a significantly better median PFS (44 months vs. 12 months) and median OS (68 months vs. 14 months). Incidence of double/triple hit MM is 4.75% in newly diagnosed MM. While majority achieve a VGPR or deeper response to induction, one-third patients progress after initial response. Our study shows the possibility of better outcomes with transplant in first remission and maintenance in this subgroup of patients, however the outcomes of ultra-high-risk subgroup in real-world clinical practice remains limited.