Chapters
- 00:00:15Patients with monoclonal gammopathy of unknown significance,
- 00:00:20smoldering multiple myeloma, or multiple myeloma,
- 00:00:25when they present with unusual features of macroglossia
- 00:00:33periorbital ecchymosis or racoon eyes,
- 00:00:36or zero negative rheumatoid arthritis,
- 00:00:41AL amyloidosis should be suspected and ruled out.
- 00:00:46Patients who present with coagulopathy
- 00:00:50related to acquired Factor X deficiency,
- 00:00:54AL amyloidosis should always be in the differential diagnosis.
- 00:01:00Patients with monoclonal gammopathy of unknown significance,
- 00:01:04smoldering myeloma or myeloma presenting with heart failure,
- 00:01:11shortness of breath, peripheral edema not related to steroids
- 00:01:16should always be assessed for AL amyloidosis.
- 00:01:20Congo red staining should be performed to rule out AL amyloidosis
- 00:01:26as bone marrow biopsies with Congo red stain
- 00:01:30can be positive in about 50% of the patients with AL amyloidosis.
- 00:01:42I think it is crucial that the subspecialties collaborate with each other,
- 00:01:48speak to each other, and see patients at the same time
- 00:01:52to make the diagnosis quicker, easier, and emergently
- 00:01:58so that the end organ function is preserved and does not deteriorate rapidly.
- 00:02:04The other specialties besides hematology oncology and cardiology
- 00:02:09are nephrology, neurology, as well as sometimes gastroenterology.
- 00:02:15But I feel that the primary care doctors are the gatekeepers of healthcare system,
- 00:02:22and raising awareness and having a high index of suspicion for a patient
- 00:02:28with multiorgan involvement, multiorgan symptoms, is critical
- 00:02:35for a primary care doctor to keep this diagnosis in mind.
- 00:02:39As a systemic disease, there’s going to be other manifestations
- 00:02:42that raise suspicion for AL cardiac amyloidosis.
- 00:02:46And I think that’s what we do so well here at Boston University
- 00:02:50where we all work together with multiple specialties,
- 00:02:54and all of us see a single patient and then we discuss that patient in a group.
- 00:03:00That also has implications for treatment, and Dr. Sanchorawala can talk more about that,
- 00:03:04but people with cardiac amyloidosis may not tolerate certain treatments as well.
- 00:03:11And so that’s really important to have a cardiologist who can talk to
- 00:03:14the hematologist about the ability of a patient to tolerate certain therapies.
- 00:03:20It is obtaining diagnosis quickly and non-invasively as possible.
- 00:03:26And then, once the diagnosis is obtained, it is crucial to collaborate,
- 00:03:31because the treatment side effects can be managed
- 00:03:34by a multidisciplinary team appropriately.