AL AMYLOIDOSIS

Question 1

AL AMYLODOSIS

Answer 1

Systemic light chain amyloidosis

Question 2

AL Amyloidosis

Answer 2

Misfolding of soluble protein(light chain)—>oligomers->aggregates->cross beta amyloid fibrils characterized by beta-sheets

Question 3

AL AMYLOIDOSIS

Answer 3

Pleated B sheets are deposited extracellularly in various organs and tissue

Question 4

Systemic Amyloidosis 2forms

Answer 4

1. AL AMYLOIDOSIS
2. Wild type transthyretin amyloidosis

Question 5

AL AMYLOIDOSIS ASSOCIATED WITH

Answer 5

Plasma cell dyscrasia

Question 6

AL AMYLOIDOSIS ASSOCIATED W PLASMA CELL DYSCRASIA

Answer 6

Plasma cell trigger excessive production of amyloidogenic immunoglobulin light chains that aggregate into oligomers and amyloid fibrils

Question 7

Plasma cell dyscrasia

Answer 7

Lambda immunoglobulin (75-80%)
Kappa light chain (20-25%)
AH amyloidosis (heavy chain)

Question 8

AL AMYLOIDOSIS CLINICAL PRESENTATION

Answer 8

1. Fatigue, weight loss
2. Nephrotic range proteinuria
3. Pleural effusion
4. GI bleeding
5. HFpEF, LVH
6. Autonomic nervous system (orthostatic hypotension)
7. Peripheral nervous system

Question 9

AL AMYLOIDOSIS DIAGNOSIS

Answer 9

1. Tissue biopsy (green birefringence when stained with Congo red)
2. Serum/urine immunofixation electrophoresis) 3. Bone marrow aspiration 4. Immunohistochemical studies 5. Mass spectrometry
3. PET W radiotracers (AL), Bone scan(atte)

Question 10

AL AMYLOIDOSIS SUPPRTIVE CARE
Orthostatic hypotension

Answer 10

1. Thigh high stockings
2. Midodrine, pyridostigmine, droxidopa

Question 11

AL AMYLOIDOSIS diarrhea

Answer 11

Loperamide, diphenoxylate-atropine,
Tincture of opium, octreotide, r/o small bowel intestinal bacterial growth