From the Guidelines
For a patient with multiple myeloma and amyloidosis experiencing shortness of breath, a comprehensive assessment plan should begin with a thorough history and physical examination, focusing on cardiac and pulmonary symptoms, and initial testing should include basic labs, chest X-ray, electrocardiogram, and pulse oximetry, as well as more specialized testing such as echocardiography and pulmonary function tests, as recommended by 1. The assessment plan should prioritize evaluating the patient's cardiac and pulmonary status, as amyloidosis can cause shortness of breath through multiple mechanisms, including heart failure from cardiac amyloid deposits, direct lung involvement, pleural effusions, or anemia from multiple myeloma.
- Initial testing should include:
- Basic labs: complete blood count, comprehensive metabolic panel, BNP or NT-proBNP
- Chest X-ray
- Electrocardiogram
- Pulse oximetry
- More specialized testing should include:
- Echocardiography to assess for cardiac amyloidosis
- Pulmonary function tests to evaluate for restrictive lung disease
- High-resolution CT scan of the chest to identify pulmonary amyloid deposits or other lung involvement
- Cardiac MRI with gadolinium enhancement and possibly a cardiac biopsy if cardiac involvement is suspected
- Bronchoscopy with biopsy if pulmonary concerns are present
- A 6-minute walk test to objectively measure exercise capacity and oxygen desaturation The patient's current multiple myeloma status should also be evaluated, as disease progression may contribute to symptoms, and treatment will depend on the underlying cause identified through this assessment, as noted in 1 and 1. It is essential to use the same test for serial studies to ensure accurate relative quantification, as mentioned in 1, and to consider the patient's individual characteristics, such as the presence of monoclonal gammopathy of uncertain significance (MGUS) or abnormal free light chains (FLCs), as discussed in 1. By following this comprehensive assessment plan, healthcare providers can effectively evaluate and manage patients with multiple myeloma and amyloidosis experiencing shortness of breath, and improve their morbidity, mortality, and quality of life outcomes.
From the FDA Drug Label
The Kansas City Cardiomyopathy Questionnaire-Overall Summary (KCCQ-OS) score is composed of four domains including Total Symptoms (Symptom Frequency and Symptom Burden), Physical Limitation, Quality of Life, and Social Limitation. Results from the F-S method represented by win ratio for the combined endpoint and its components (all-cause mortality and frequency of CV-related hospitalization) consistently favored VYNDAQEL versus placebo across all subgroups (wild-type, variant and NYHA Class I & II, and III), except for CV-related hospitalization frequency in NYHA Class III
The patient's shortness of breath can be assessed using the 6-Minute Walk Test (6MWT) and the Kansas City Cardiomyopathy Questionnaire-Overall Summary (KCCQ-OS) score.
- The 6MWT measures the distance a patient can walk in 6 minutes, which can indicate functional capacity.
- The KCCQ-OS score assesses the patient's symptoms, physical limitation, quality of life, and social limitation. These tests can help evaluate the patient's condition and guide the assessment plan 2. Key components of the assessment plan:
- Medical history: Review the patient's medical history, including multiple myeloma and amyloidosis.
- Symptom assessment: Evaluate the patient's symptoms, including shortness of breath, using the KCCQ-OS score.
- Functional capacity: Assess the patient's functional capacity using the 6MWT.
- Cardiovascular evaluation: Evaluate the patient's cardiovascular status, including frequency of CV-related hospitalization.
From the Research
Assessment Plan for Shortness of Breath in Multiple Myeloma and Amyloidosis
To assess shortness of breath in a patient with multiple myeloma and amyloidosis, the following tests and evaluations can be considered:
- Echocardiography to evaluate cardiac function and detect signs of restrictive cardiomyopathy (RCM) or cardiac amyloidosis 3, 4
- Cardiac MRI to further evaluate cardiac structure and function 3
- Fat pad biopsy or other tissue biopsies (e.g., pleural, pericardial, or lung biopsy) to confirm the presence of amyloid deposits 3, 5, 6
- Bone marrow biopsy to assess plasma cell percentage and diagnose multiple myeloma 3, 4
- Skeletal survey to detect lytic bone lesions 3
- Serum electrophoresis to identify monoclonal protein 4
- Pleural fluid test and thoracentesis to evaluate pleural effusions 5
- Endomyocardial biopsy with mass spectrometry to differentiate between light chain amyloidosis and transthyretin cardiac amyloidosis 7
Differential Diagnoses
When evaluating shortness of breath in a patient with multiple myeloma and amyloidosis, consider the following differential diagnoses:
- Restrictive cardiomyopathy (RCM) due to amyloid deposition 3
- Cardiac amyloidosis 3, 4
- Pleural and pericardial effusions due to amyloidosis 5
- Pulmonary amyloidosis 6
- Transthyretin cardiac amyloidosis 7
Importance of Accurate Diagnosis
Accurate diagnosis is crucial in patients with multiple myeloma and amyloidosis, as it guides treatment and management decisions 3, 5, 4, 7. A comprehensive assessment plan, including a combination of imaging studies, biopsies, and laboratory tests, can help establish an accurate diagnosis and improve patient outcomes.