AL Amyloidosis and Multiple Myeloma: A Complex Scenario in Which Cardiac Involvement Remains the Key Prognostic
Rafael Ríos-Tamayo1, Isabel Krsnik1, Manuel Gómez-Bueno1
1Hospital Universitario Puerta de Hierro, IDIPHISA, CIBERCV, 28222 Majadahonda, Spain.
Insights
Systemic light chain amyloidosis (AL) and multiple myeloma (MM) co-occurrence was studied in 141 patients. The AL/MM association appeared synchronous and impacted survival, though not significantly, highlighting the need for further research.
Area of Science:
- Hematology
- Oncology
- Cardiology
Background:
- Monoclonal gammopathies (MGs) can evolve, with comorbidities like co-occurring MGs being significant.
- The association between systemic light chain (AL) amyloidosis and multiple myeloma (MM) lacks extensive evidence.
Purpose of the Study:
- To investigate the characteristics and prognostic impact of co-occurring AL amyloidosis and multiple myeloma (AL/MM).
Main Methods:
- Prospective enrollment of 141 AL amyloidosis patients from January 2005 to April 2023.
- Assessment of diagnostic criteria for multiple myeloma (MM) in AL amyloidosis patients.
- Survival analysis comparing AL/MM patients with AL-only patients.
Main Results:
- 18.7% of AL amyloidosis patients met criteria for MM (AL/MM).
- Synchronous AL/MM was more frequent than sequential occurrence.
- AL/MM patients showed a trend towards poorer overall survival, but the difference was not statistically significant.
- Age, NT-proBNP levels, and autologous stem cell transplant were independent prognostic factors in AL amyloidosis.
Conclusions:
- The AL/MM association is observed in a significant subset of AL amyloidosis patients.
- While not statistically significant in this series, the AL/MM association warrants further investigation due to potential survival implications.
- Cardiac involvement remains a dominant prognostic factor in AL amyloidosis, irrespective of MM co-occurrence.
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.
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