108 - Amyloidosis

Question 1

What does AL amyloidosis stand for? What is the clinical syndrome related to it?

Answer 1

Amyloidosis of Light chain

Primary or myeloma associated

Question 2

What does AA amyloidosis stand for?

Answer 2

Amyloidosis of serum amyloid A protein

Secondary; reactive

Question 3

To which group of amyloidosis do AL and AA belong?

Answer 3

Systemic amyloidosis

Question 4

The clinical symptoms of amyloidosis are ____

Answer 4

Organ dependent (Renal, Cardiac, Endo….)

Question 5

How would you diagnose amyloidosis? (4)

Answer 5

1. Suspect patients with multiorgan dysfunction (nephropathy, cardiomyopathy, neuropathy
2. Look for pathognomonic findings (macroglossia, periorbital ecchymoses)
3. Obtain noninvasive biopsy (visceral fat)
4. if - consider sampling the damage organ

Question 6

What are the lab changes you may find in amyloidosis? (6)

Answer 6

1. Normal CBC
2. Increased ESR
3. Proteinuria
4. Albuminemia
5. BNP/ pro-BNP/ troponin increase
6. Cholestasis + ALP increase

Question 7

The most common etiology of AL amyloidosis is ___ of plasma cells from the ___ secreting ____ light chains which deposit as ____ in the tissues

Answer 7

Clonal spread
Bone marrow
Monoclonal immunoglobulin
Amyloid fibril

Question 8

Other syndrome that may lead to AL amyloidosis include: (2)

Answer 8

1. Non Hodgkin’s lymphoma

2. Waldenstorm macroglobulinemia

Question 9

The common light chain in AL amyloidosis is

Answer 9

Lambda

Question 10

The most common organ involved in AL amyloidosis is ___, followed by ___ which is the most common factor for ____

Answer 10

The kidney (70-80%)
The heart (50-60%)
Mortality

Question 11

Treatment for AL amyloidosis includes ____ and ___ which will decrease the number of ____, but only rarely lead to ___

Answer 11

Oral Melphalan
Prednisone/ dexamethasone
Plasma cells
Full remission

Question 12

What will you prescribe a post bone marrow AL amyloidosis patient

Answer 12

IV Melphalan

Question 13

What is the best treatment for AL amyloidosis patients with heart involvement?

Answer 13

Heart transplantation

Question 14

Untreated AL amyloidosis has ____ from the time of diagnosis and ____ with ____ symptoms

Answer 14

1-2 years
6 months
Cardiac

Question 15

AA amyloidosis can occur with any ____ such as (3) or ____. It is also related to ___, ___, ____.

Answer 15

Chronic inflammation
(RA, IBD, FMF)
Chronic infection (TB)
Castleman
Lymphoma
RCC