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Chapters

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