Chapter 47 - Amyloidosis Flashcards Preview

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Flashcards in Chapter 47 - Amyloidosis Deck (14):

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


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

Macroglossia together with carpal tunnel syndrome


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)


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



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



What's frictional amyloidosis?

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


What are the 3 major forms of primary cutaneous amyloidosis?

1) Macular, 2) lichen, 3) nodular


What is the common feature in all types of amyloidosis?

Abnormal extra-cellular deposition of amyloid in tissues


What are the 2 main types of amyloidosis?

Systemic, organ-limited/localized


What are the 3 types of systemic amyloidosis?

1) primary, 2) secondary, 3) heredofamilial


What are the 3 types of organ-limited amyloidosis?

1) cutaneous, 2) endocrine, 3) cerebral


What are some special stains for amyloid?

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


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


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