Category Ranking

98%

Total Visits

921

Avg Visit Duration

2 minutes

Citations

20

Article Abstract

Monoclonal gammopathies (MGs) are a wide range of diseases that may evolve or progress over time. Comorbidity plays a critical role in this setting. The co-occurrence of two MGs is not a rare event. The evidence on the association of systemic light chain (AL) amyloidosis and multiple myeloma (MM) is scarce and controversial. Herein we aim to address this topic in a large series of patients of a referral center. All consecutive AL amyloidosis patients treated at our center from January 2005 to April 2023 were prospectively enrolled in a clinical and epidemiological registry. 141 patients diagnosed with AL amyloidosis were included, of which 7 (5%) had localized whereas 134 presented with systemic disease. The heart was the most frequently affected organ (90.3%). 25 patients (18.7%) fulfilled the IMWG diagnostic criteria of MM (AL/MM). Time-dependent association between AL and MM showed that the synchronous pattern is more frequent than the appearance of a second primary malignancy. The diagnostic delay was six months (m). Patients with AL/MM had a poorer median overall survival (OS) than AL-only patients (35.5 m, CI 95% 0-88.9, vs. 52.6 m, CI 95% 16.7-88.5), but this difference was not statistically significant. The prognosis in AL is dominated by the heart involvement, which is massive in this series. In our Cox regression model, only three prognostic variables remain as independent prognostic factors: age, N-terminal pro-brain natriuretic peptide (≥8500 ng/L), and undergoing an autologous stem cell transplant, whereas left ventricular ejection fraction shows a marginal effect. More and large studies focusing on the AL/MM association are needed to uncover the characteristics and prognostic impact of this association.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10382070PMC
http://dx.doi.org/10.3390/life13071518DOI Listing

Publication Analysis

Top Keywords

amyloidosis multiple
8
multiple myeloma
8
patients
6
amyloidosis
4
myeloma complex
4
complex scenario
4
scenario cardiac
4
cardiac involvement
4
involvement remains
4
remains key
4

Similar Publications

Clinical Alzheimer's disease is currently characterized by cerebral β-amyloidosis associated with cognitive impairment. However, most cases of Alzheimer's disease are associated with multiple neuropathologies at autopsy. The peripheral protein changes associated with these disease endophenotypes are poorly understood.

View Article and Find Full Text PDF

IntroductionDaratumumab is a therapeutic cornerstone of the management of multiple myeloma, exerting its anti-myeloma activity through targeting of the cell surface glycoprotein CD38 on plasma cells. While originally given intravenously, the subcutaneous formulation, daratumumab hyaluronidase injection (Dara SC), has been associated with non-inferior efficacy and lower infusion-related reaction rates (IRRs) in the treatment of multiple myeloma and light chain amyloidosis. A noted benefit of Dara SC is a short administration time; however, the optimal observation time post injection to ensure patient safety is unclear from the drug labeling.

View Article and Find Full Text PDF

Isatuximab is an IgG1k monoclonal antibody that binds with high affinity to CD38 expressed on plasma cells. Anti-CD38 antibodies have shown efficacy as monotherapy and in combination in a variety of settings for patients with multiple myeloma and light chain (AL) amyloidosis. This multi-center, cooperative group phase 2 trial was designed to evaluate hematologic response, organ response, and safety of isatuximab monotherapy for the treatment of relapsed AL amyloidosis.

View Article and Find Full Text PDF