Chapter 47 - Amyloidosis Flashcards Preview

Bolognia Book Club > Chapter 47 - Amyloidosis > Flashcards

Flashcards in Chapter 47 - Amyloidosis Deck (14):
1

AA protein (amyloid A protein) is the protein that accumulates in secondary systemic amyloidosis. What's the precursor for this amyloid?

An acute pause protein which is synthesized by the liver and appears to have a regulatory function in lipoprotein metabolism during inflammation

2

What's the classic clinical presentation of primary systemic amyloidosis? List two clinical findings.

Macroglossia together with carpal tunnel syndrome

3

What's the "raccoon sign" as it relates to primary systemic amyloidosis?

Periorbital ecchymoses due to minor trauma, such as precipitated by coughing, the Valsalva maneuver, proctoscopy for a rectal biopsy, or after pinching or rubbing the skin (pinch purpura)

4

What percentage of patients with primary systemic amyloidosis have clinically apparent skin involvement?

25%

5

True or false: primary systemic amyloidosis is usually associated with an underlying plasma cell dyscrasia.

True

6

What's frictional amyloidosis?

It describes amyloidosis produced by friction from nylon brushes, towels, and other rough materials

7

What are the 3 major forms of primary cutaneous amyloidosis?

1) Macular, 2) lichen, 3) nodular

8

What is the common feature in all types of amyloidosis?

Abnormal extra-cellular deposition of amyloid in tissues

9

What are the 2 main types of amyloidosis?

Systemic, organ-limited/localized

10

What are the 3 types of systemic amyloidosis?

1) primary, 2) secondary, 3) heredofamilial

11

What are the 3 types of organ-limited amyloidosis?

1) cutaneous, 2) endocrine, 3) cerebral

12

What are some special stains for amyloid?

Congo red, crystal violet, methyl violet, PAS, Sirius red, pagoda red, Dylon stain, thioflavin T

13

What is the treatment for primary cutaneous amyloidosis?

No curative treatment. Aimed at breaking itch-scratch cycle, potent topical steroids, mild keratolytic agent, occlusive dressing, topical calcineurin inhibitor, phototherapy, dermabrasion, PO retinoids

14

What is the treatment for primary systemic amyloidosis?

In young patients, high dose melphalan, followed by autologous peripheral blood stem cell transplant. In elderly patients, melphalan + prednisone + thalidomide/lenalidomide/bortezomib. Both groups also require supportive tx depending on organ involvement