Light chain multiple myeloma, clinic features, responses to therapy and survival in a long-term study

Abstract

BackgroundWe intended to investigate the long-term clinical characteristics, responses to therapy and survival in patients with lightchain multiple myeloma (MM).MethodsNinety-six patients were enrolled into the study. There were 42 κ-chain MM patients and 54 λ-chain MM patients. All the patients werestage III in the Durie-Salmonstaging system. Among them, 66 patients received Velcade (bortezomib) treatment and the other 30 did not.ResultsThe main symptoms of these patients included bone pain (77.1%), weakness and fatigue (12.5%), foamy urine (8.3%) and extramedullaryplasmocytomas (33.3%). The overall response rate (ORR) was 95.5% in patients treated with Velcade and 60%in the patients without. The median survival times were 23 months in patients treated with Velcade and 12 months in patients without. The median time of progression-free survival (PFS) was nine months in patients treated with Velcade and five months in patients without. The one-year PFS and two-year PFS were 37% and 25%, 27% and 9% for patients treated with Velcade, or without, respectively. The three-year overall survival (OS) and five-year OS were 33% and 24%, 28% and 9% for patients treated with Velcade, or without, respectively. There was no significance in OS between the two groups (P = 0.335). But there was significant difference in PFS between the two groups (P = 0.036).ConclusionsOur long-term study demonstrated that patients with lightchain myeloma appeared to have more aggressive disease courses and poor outcomes, which could be improved by treatment with Velcade.

Cancer/Testis Antigen Expression Panel Incorporating MAGEC1 and BAGE2 Predicts Multiple Myeloma Disease Stage and Severity

“…This could possibly indicate that disease progression could be enumerated by the number of CTAs expressed in this panel and could be used to re-evaluate patients if disease progression is suspected. Only a small group of patients represented either the IgA or light chain disease subtypes, both of which are associated with a worse overall survival [33,34]. While no significant associations could be found regarding the IgA grouping, due to low numbers, the light chain diseased patients all grouped together expressing 5-6 CTAs and demonstrated advanced clinical symptoms.…”

“…The characteristics of the plasma cells, including their presence in the PB, proliferation status and their abnormal nuclear staining are additional independent prognostic factors that can be considered. Circulating plasma cells, which is indicative of more aggressive disease [33,34] were only observed in 9 patients, 78% of whom presented with stage III disease. However, this subgrouping showed no specific CTA expression pattern (in number or specific gene).…”

“…This association was further strengthened by the increase in the number of CTAs expressed by two AMM patients who progressed to MM within 12 months, both showing no CTA expression at initial diagnosis and 3 (stage II) and 6 (stage III) genes, respectively at follow up. With regards to disease subtype, light chain MM disease is known to offer a worse prognosis [32,33], with rapid kidney failure. While we only had 5 patients in this grouping at diagnosis, all presented with advanced disease (stage III, 3-4 CRAB symptoms) and correspondingly expressed 5-6 CTAs (Figure 2).…”

Clinical characteristics of pain syndrome in patients with multiple myeloma

“…В опубликованных работах, посвященных проблемам ММ, практически не обсуждалась локализация болей у пациентов, указывалась только общая частота болевого синдрома [19][20][21]. В нашем исследовании наиболее часто встречалась множественная локализация болей (38,9%), следовательно, наличие алгического синдрома множе-ственной локализации может служить ранним признаком миеломной болезни и должно побуждать врачей к ее диа-гностическому поиску.…”

The challenges of checkpoint inhibition in the treatment of multiple myeloma

“…Production of this aberrant Ig results in several of the complications associated with MM such as renal dysfunction, neuropathy, and hyperviscosity syndrome 23 . However, in approximately 15–20% of patients the abnormal plasma cells secrete only monoclonal free light chains, and in approximately 2–3% of cases these cells secrete no monoclonal protein at all, resulting in the so-called non-secretory myeloma 45 . Myeloma cell growth in the bone marrow and the resultant cytokines produced by these transformed cells lead to the classic symptoms of active MM: osteolytic bone lesions, hypercalcemia, and anemia 6 .…”