Amyloidosis

Amyloidosis

Amyloidosis

Which regimens have been shown to have activity r/r amyloidosis ?

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Pd, Rd, CPd, BD, bortezimib, CRd, CTd, daratumumab, Ixazomib-Dex

Amyloidosis
Amyloidosis

Which regimens have phase 3 randomized data supporting their used in r/r amyloidosis ?

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None, all phase 2

Amyloidosis
Amyloidosis

Is there activity with daratumumab in AL amyloidosis ?

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Yes

Amyloidosis
amyloidosis

How do you manage familial ATTR amyloidosis?

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Liver transplant with or without cardiac transplant even if there is no evidence of disease

amyloidosis
amyloidosis

What is the treatment for localized light chain amyloidosis with no evidence of systemic disease?

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Surgery and resection

amyloidosis
amyloidosis

Which FISH translocation shows a worse response to bortezomib in light chain amyloidosis?

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t(11;14)

amyloidosis
Amyloidosis

What is the difference between AL and AA amyloidosis?

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AL- immunoglobulin light chain fragment as part of a plasma cell dyscrasia
AA- amyloid as an acute phase reactant of chronic inflammation

Amyloidosis
Amyloidosis

Which cofactor protein is associated with amyloidosis?

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Apolipoprotein E

Amyloidosis
Amyloidosis

What is the structural protein arrangement in amyloidosis?

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Anti-parallel

Amyloidosis
Amyloidosis

What is the definition of an amyloid?

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Low molecular weight protein subunits from various different proteins deposited in the extracellular
matrix of various tissues

Amyloidosis
Amyloidosis

Which stain is needed for amyloidosis diagnosis?

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Congo red with polarized light showing Apple Green birefringence

Amyloidosis
Amyloidosis

What amyloid associated end-stage renal disease should be considered on a differential diagnosis ?

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Dialysis related amyloidosis

Amyloidosis
Amyloidosis

What 3 cancers are commonly associated with amyloidosis?

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Multiple myeloma, Waldenstrom’s, non-Hodgkin’s lymphoma

Amyloidosis
Amyloidosis

What is the difference between light chain disease and AL?

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No fibrils and no concurrent amyloid deposits with light chain disease

Amyloidosis
Amyloidosis

What is the most common cause of AA amyloidosis?

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Rheumatoid arthritis

Amyloidosis
Amyloidosis

Which protein is the culprit for dialysis-related amyloidosis?

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Beta-2 microglobulin

Amyloidosis
Amyloidosis

What is a common tissue target in dialysis-related amyloidosis?

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Osteoarticular strictures

Amyloidosis
Amyloidosis

What is a common presentation in dialysis-related amyloidosis?

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Rheumatoid arthritis

Amyloidosis
Amyloidosis

Which protein is seen in hereditary amyloidosis?

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Mutated transthyretin – prealbumin

Amyloidosis
Amyloidosis

What is the senile, age-related systemic amyloidosis?

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Unmutated Transthyretin deposition but low clinical consequence

Amyloidosis
Amyloidosis

What are are some examples of single tissue nonsystemic amyloid disease?

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Alzheimer’s, skin only, bladder only, liver only
Skin only – associated with multiple endocrine neoplasia
Bladder only -associated with immunoglobulin light chain
Liver only – associated to LECT2

Amyloidosis
Amyloidosis

Which end-organs get the highest AL burden?

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Kidney, heart and liver

Amyloidosis
Amyloidosis

Which types of amyloidosis can cause kidney disease, cardiac disease, gastrointestinal disease?

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Kidney–AA and AL
GI- AA and AL
Cardiac-ATTR and AL

Amyloidosis
Amyloidosis

What are some central nervous system diseases that amyloidosis can cause?

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Stroke, neuropathy, dementia, Alzheimer’s

Amyloidosis
Amyloidosis

What are the pulmonary manifestations of amyloidosis?

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Tracheobronchial infiltration, pleural effusion, parenchymal nodules, pulmonary hypertension

Amyloidosis
Amyloidosis

What are some hematologic consequences of amyloidosis?

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Bleeding from the low factor X levels
Anemia secondary to kidney involvement
Thrombocytopenia because of an enlarged spleen

Amyloidosis
Amyloidosis

What develops around the eyes of patients with amyloidosis?

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Raccoon eyes

Amyloidosis
Amyloidosis

What multiple myeloma presentation should make you suspicious about amyloidosis?

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Nephrotic syndrome

Amyloidosis
Amyloidosis

What are the 4 main categories of amyloidosis therapy?

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AL–multiple myeloma regimens
AA–anti-inflammatory regimens
End-stage renal disease related amyloidosis – change the dialysis method or consider kidney transplant
Liver related amyloidosis- liver transplant

Amyloidosis
Amyloidosis

What is the advantage of a fat pad biopsy in amyloidosis?

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Low bleeding risk compared to kidney or liver or rectal biopsy

Amyloidosis
Amyloidosis

When should a visceral organ biopsy be opted for versus a fat-pad biopsy for amyloidosis diagnosis?

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If the complication risk is lower, very accessible lesion

Amyloidosis
Amyloidosis

What workup is required for a new diagnosis of amyloidosis without a history of infection or
autoimmune disease?

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Multiple myeloma / Lymphoma workup because almost always a plasma cell dyscrasia

Amyloidosis
Amyloidosis

What imaging modality is available for amyloidosis ?

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Serum amyloid P (SAP) component

Amyloidosis
Amyloidosis

What defines high risk light chain amyloidosis with respect to transplant?

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cTnT greater than 0.035
NT-pro-BNP greater than 332 ng/L

Amyloidosis
Amyloidosis

What is the first line treatment in a non-transplant eligible patient with amyloidosis, light chain?

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Melphalan plus dexamethasone or bortezomib-based regimen

Amyloidosis
Amyloidosis

What is the 1 year mortality for amyloidosis patients with 2 of the following risk factors ?

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Cardiac troponin greater than 0.01 mcg/L
NT-Pro-BNP greater than 4200 ng/L
Uric acid greater than 8.0 mg/dL
80%

Amyloidosis
Amyloidosis

Which serum markers are used as prognostic variables for amyloidosis?

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NT-pro-BNP (>332)
Troponin greater than 0.035
Uric acid greater than 8.0
Kappa, lambda light chain greater than 18 mg/dL

Amyloidosis
Amyloidosis

What are the 3 types of deposition disease?

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Amyloidosis, light chain deposition disease, heavy chain deposition disease

Amyloidosis
Amyloidosis

What is the 10% rule for amyloidosis–myeloma?

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Amyloidosis incidence is 10% of multiple myeloma incidence
Amyloidosis patients have a 10% concurrent multiple myeloma diagnosis

Amyloidosis
Amyloidosis

Which cardiac tests are required at diagnosis for amyloidosis?

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BNP, troponin T, NT-pro-BNP

Amyloidosis
Amyloidosis

What laboratory cut-offs are used for autologous stem cell transplant eligibility for amyloidosis?

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Troponin less than 0.06 ng/mL
NT-pro-BNP less than 5000 ng/mL

Amyloidosis
Amyloidosis

What unique laboratory changes predict for 50% mortality in amyloidosis pretransplant?

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Factor X level less than 25%

Amyloidosis
Amyloidosis

How do you manage factor X levels less than 25% pre-transplant for amyloidosis?

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Splenectomy

Amyloidosis
Amyloidosis

What is the difference in 4 year overall survival with autologous stem cell transplant versus
chemotherapy and amyloidosis?

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70% versus 40%.

Amyloidosis
Amyloidosis

What melphalan dose is required for amyloidosis stem cell transplant?

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200 mg/m2 because poor survival outcomes with lower dose

Amyloidosis
Amyloidosis

What melphalan regimen is used for nontransplant amyloidosis?

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Oral melphalan day 1-4 on the 28 day schedule plus dexamethasone 40 mg day 1-4 on a 28 day schedule

Amyloidosis
Amyloidosis

What drugs can be used for relapsed or refractory amyloidosis?

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All the multiple multiple myeloma regimens

Amyloidosis
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