From the Guidelines
Inspiratory spirometry is not a recommended routine practice in the management of AL amyloidosis, and treatment should focus on addressing the underlying plasma cell dyscrasia with therapies such as daratumumab-CyBorD, which has emerged as the standard of care for newly diagnosed AL amyloidosis. AL amyloidosis patients often develop respiratory complications due to amyloid protein deposition in the lungs, pleura, and diaphragm, leading to restrictive lung disease and reduced lung capacity 1. While inspiratory spirometry is commonly used in post-operative settings to prevent atelectasis and pneumonia, there is no specific evidence supporting its routine use in AL amyloidosis.
Management should instead focus on treating the underlying plasma cell dyscrasia with chemotherapy regimens such as bortezomib-based combinations (CyBorD: cyclophosphamide, bortezomib, and dexamethasone) or daratumumab-based therapies, as supported by the landmark ANDROMEDA study, which demonstrated an unprecedented high rate of deep hematologic responses with daratumumab plus CyBorD 1. For patients with significant respiratory involvement, standard pulmonary supportive care including:
- Supplemental oxygen
- Pulmonary rehabilitation
- Treatment of infections is appropriate. Respiratory function should be monitored regularly with pulmonary function tests to assess disease progression and treatment response. Any respiratory interventions should be tailored to the individual patient's specific manifestations and complications of amyloidosis rather than applying inspiratory spirometry as a standard approach.
From the Research
Treatment Options for AL Amyloidosis
- The combination of cyclophosphamide, bortezomib, and dexamethasone (CVD) has been shown to be effective in treating AL amyloidosis, with an overall hematologic response rate of 81.4% 2.
- The use of cyclophosphamide, bortezomib, and methylprednisolone (CyBorMe) has also been explored, with promising results, including a faster time to first response and a higher cardiac response rate compared to CVD 3.
- Bortezomib and dexamethasone pre- and post-risk-adapted autologous stem cell transplantation has been shown to be a safe and effective approach, yielding deep and durable remissions and promoting organ recovery 4.
Diagnosis and Management
- Amyloidosis is a disorder of protein folding, and AL amyloidosis can arise as a consequence of chronic respiratory conditions 5.
- Pulmonary AL amyloidosis can be localized or associated with systemic involvement, and presentation can vary from clinically silent to severe 6.
- Diagnostic workup requires tissue biopsy and identification by immunohistochemical staining, and systemic treatment has evolved to include combination therapies such as daratumumab, bortezomib, cyclophosphamide, and dexamethasone (dara-VCD) as first-line therapy 6.
Response Rates and Survival
- Patients treated with CVD upfront had higher rates of complete response (65.0%) and very good partial response (66.7%) compared to those treated at relapse 2.
- The estimated 2-year progression-free survival was 66.5% for patients treated upfront and 41.4% for relapsed patients 2.
- Patients achieving minimal residual disease negative complete response had superior progression-free survival (P= .008) 4.