Chapter 47 - Amyloidosis

Question 1

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

Answer 1

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

Question 2

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

Answer 2

Macroglossia together with carpal tunnel syndrome

Question 3

What’s the “raccoon sign” as it relates to primary systemic amyloidosis?

Answer 3

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)

Question 4

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

Answer 4

25%

Question 5

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

Answer 5

True

Question 6

What’s frictional amyloidosis?

Answer 6

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

Question 7

What are the 3 major forms of primary cutaneous amyloidosis?

Answer 7

1) Macular, 2) lichen, 3) nodular

Question 8

What is the common feature in all types of amyloidosis?

Answer 8

Abnormal extra-cellular deposition of amyloid in tissues

Question 9

What are the 2 main types of amyloidosis?

Answer 9

Systemic, organ-limited/localized

Question 10

What are the 3 types of systemic amyloidosis?

Answer 10

1) primary, 2) secondary, 3) heredofamilial

Question 11

What are the 3 types of organ-limited amyloidosis?

Answer 11

1) cutaneous, 2) endocrine, 3) cerebral

Question 12

What are some special stains for amyloid?

Answer 12

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

Question 13

What is the treatment for primary cutaneous amyloidosis?

Answer 13

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

Question 14

What is the treatment for primary systemic amyloidosis?

Answer 14

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