21 - 125 - AMYLOIDOSIS

Question 1

Which of the following is included in the Histologic Criteria for the definition of Amyloid?

A. Heterogenous-Hyaline

B Mostly intracellular

C. Typical 10-15 nm fibrils

D. Alcian blue positive

Answer 1

C. Typical 10-15 nm fibrils

Question 2

The following are included in the organ system involvement for AL amyloidosis except:

A Cardiac

BHepatic

C. Renal

D. Neurologic

Answer 2

D

Question 3

Localized cutaneous amyloidoses are often restricted to the ____________ .

A. Epidermis

B. Papillary dermis

C. Reticular dermis

D. Dermo-epidermal junction

Answer 3

B. Papillary dermis

Question 4

The most common type of cutaneous amyloidosis is ____________ .

A. Macular amyloidosis

B. Lichen amyloidosis

C. Biphasic cutaneous amyloidosis

D. Systemic amyloidosis

Answer 4

B. Lichen amyloidosis

Question 5

Which of the following presents with 1 or more d seated lesions that have a waxy or nodular appearance? They can appear as papules, nodules, or plaques. Common sites include the feet and trunk.

A. Lichen amyloidosis

B. Amyloid adjacent to tumor

C. Nodular amyloidosis

D. Transthyretin amyloidosis

Answer 5

C. Nodular amyloidosis

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Question 28

Stains for amyloid. describe the findings per stain

Answer 28

* Congo red - metachromasia and apple-green birefringence * Thioflavin T - yellow-green fluorescence

Question 29

Ultrastructurally,what are the main constituents of amyloid deposits?

Answer 29

fibrils

Question 30

second feature of all amyloid deposits is the presence of what?

Answer 30

pentraxin, serum amyloid P-component (SAP)

Question 31

features unique to AL and Aβ2m

Answer 31

Macroglossia, lingual nodules, or lateral ridging

Question 32

A common and often diagnostic feature of AL amyloidosis in histopath

Answer 32

presence of amyloid in the walls of small or medium-sized blood vessels, causing vascular wall weakening and hemorrhage.

Question 33

Macular amyloidosis commonly presents in what area?

Answer 33

interscapular region

Question 33

cutis laxa is a major manifestation of what type of systemic amyloidosis

Answer 33

AGel

Question 34

A useful clue (as in lichen amyloidosis) in histopath for the presence of amyloid deposits

Answer 34

presence of rare** melanin-containing histiocytes encircling these deposits**

Question 35

most common type of cutaneous amyloidosis

Answer 35

Lichen amyloidosis

Question 36

initial symptom of lichen amyloidosis

Answer 36

intense pruritus that may improve with sun exposure and worsen during periods of stress

Question 37

amyloid type of the large majority of cases of nodular amyloidosis

Answer 37

AL

Question 38

major cytokeratin constituent of amyloid deposits and suggested a role of keratinocyte apoptosis in pathogenesis.

Answer 38

CK5

Question 39

Agents that may be active as fibril disrupters that have progressed to clinical testing

Answer 39

* doxycycline * pigallocatechin3-gallate (green tea) * curcumin