AMY DA site - i can identify - Video 4


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