AMYLOIDOSIS Flashcards
Techniques used for diagnosis in suspected cases of AL amyloidosis due to monoclonal plasma cell proliferations
- Bone marrow biopsy
- Serum immunoelectrophoresis (SIEP) and urine immunoelectrophoresis (UIEP)
Type of amyloid deposited in the condition described below:
a. Armenian, Sephardic Jewish, and Arabic origins
b. attacks of fever
c. inflammation of serosal surfaces
AA
- Major amyloid fibril protein associated with chronic inflammatory conditions?
- What is the chemically related precursor protein?
- AA
- SAA
most common clinical presentaion of hemodialysis associated amyloidosis
carpal tunnel syndrome
List 5 examples of conditions associated with deposition of AA amyloid?
- Rheumatoid arthritis
- Heroin abusers (sc injection)
- Crohn disease
- solid tumors- renal cell carcinoma and Hodgkin lymphoma.
- Chronically infected burns, decubitus ulcers
Appearance of amyloid on light microscopy on an H & E section
amorphous, eosinophilic, hyaline, extracellular
- Major amyloid fibril protein associated with isolated atrial amyloidosis
- What is the chemically related precursor protein?
- AANF
- Atrial natriuretic factor
- Special stain for amyloid
- Describe the appearance of amyloid when stained with Congo Red under an ordinary light microscope
- Congo Red
- pink or red color to amyloid deposits
Most common location for deposition of amyloid in the form associated with plasma cell proliferation
perivascular and vascular deposits
- Major amyloid fibril protein associated with hemodialysis?
- What is the chemically related precursor protein?
- Aβ2M
- β2-microglobulin
- Major amyloid fibril protein associated with Type 2 diabetes
- What is the chemically related precursor protein?
- AIAPP
- Islet amyloid peptide
Amyloid deposition is intracellular or extracellular?
Extracellular
Primary underlying mechanism in pathogenesis of amyloidosis
Protein misfolding
Amyloidosis of the spleen primarily involving the splenic follicles, producing tapioca-like granules
Sago spleen
Amyloidosis of the spleen primarily involving the walls of the splenic sinuses and connective tissue framework
Lardaceous spleen
First site of deposition of amyloid
adjacent to basement membranes.
Nuclear imaging technique used to diagnose amyloidosis
Scintigraphy with radiolabeled serum amyloid P (SAP)
See the attached image. List the expected clinical consequences.
Proteinuria, nephrotic syndrome→ pitting edema/effusions; ↓oncotic pressure (↓albumin)
Renal failure
- Major amyloid fibril protein associated with multiple myeloma and other monoclonal plasma proliferations
- What is the chemically related precursor protein ?
- AL
- Immunoglobulin light chains, chiefly lambda type.
- Major amyloid fibril protein associated with familial amyloidotic polyneuropathies
- What is the chemically related precursor protein?
- ATTR
- Transthyretin
Identify the type of amyloid likely to be deposited in the condition described below:
Elderly; male
Diastolic heart failure
arrhythmias
wild-type “senile” TTR amyloid
Describe the appearance of amyloid when stained with Congo Red and viewed under polarizing light
apple-green birefringence
3 points to describe the physical nature of amyloid as seen on electron microscopy
Rigid, continuous, nonbranching fibrils
approximately 7.5 to 10 nm diameter
cross-β-pleated sheet conformation
Common sites of biopsy in systemic amyloidosis
omental fat pad, rectum, and gingiva
Why does AL amyloidosis of vessels cause hemorrhage?
AL amyloid binds and inactivates factor X
- Major amyloid fibril protein associated with medullary carcinoma thyroid
- What is the chemically related precursor protein?
- A Cal
- Calcitonin
See the attached image and description below. Identify the type of amyloid likley to be deposited.
90 year old male with dementia
Alzheimer disease - Type of amyloid fibril protein is Aβ
Precursor proteinassociated with cerebral amyloid deposits in Alzheimer disease
Amyloid precursor protein (APP)
