Amyloidosis : Definition, Pathogenesis, Classification, Symptoms, Treatment And MCQs for NEET, GPAT, CSIR NET JRF

Amyloidosis : Definition, Pathogenesis, Classification, Symptoms, Treatment And MCQs for NEET, GPAT, CSIR NET JRF

Amyloidosis is the term used for a group of disease characterized by extracellular deposition of fibrillar insoluble proteinaceous substance called amyloid.

“Amyloid is an pathogenic proteinaceous substance, deposited between cells in various tissues and organs of the body in a wide variety of clinical settings.”

“Amyloid stains positive with Congo red staining and shows apple – green birefringence on polarizing microscopy.”

PATHOGENESIS :-

Amyloidogenesis in general in vivo, occurs in following sequence :

  • A pool of amyloidogenic precursor protein
  • A nidus for fibrillogenesis
  • Partial degradation or proteolysis
  • Non – fibrillar components

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The above image is taken for education purpose only from Harsh Mohan, 7th edition; Page no. 69

CLASSIFICATION :-

Amyloidosis is classified in number of ways :

1.} Based on cause, into Primary and Secondary amyloidosis.

2.} Based on extend of amyloid deposition , into Systemic involving multiple organs and Localized involving on e or two organs or sites.

3.} Based on clinical location, into Pattern I (involving tongue, heart, bowel, skeletal and smooth muscle, skin and nerves), Pattern II (principally involving liver, spleen, kidney and adrenal) and Mixed pattern (involving sites of both Pattern I and Pattern II).

4.} Based on precursor biochemical proteins, into specific type of serum amyloid protein.

SYMPTOMS :-

Signs and symptoms of amyloidosis are as follows :

  • Swelling occur in ankles and legs
  • Bloody diarrhea or constipation
  • There will be shortness in breath
  • Fatigue and weakness
  • Rippled at edge and enlarged tongue
  • Purplish patches around the eyes
  • Difficulty in swallowing and irregular heartbeat
  • Weight loss

Amyloidosis Foundation

The above image is taken for education purpose only from amyloidosis.org

TREATMENT :-

As such there is no cure for amyloidosis, but the signs and symptoms of this disease can be controlled by certain medications.

The above image is taken for education purpose only from amyloidosis.org.uk

 

MULTIPLE CHOICE QUESTIONS :-

1.] Secondary amyloidosis is associated with ?

a. AC

b. AA

c. AL

d. APrP

2.] The best investigation foe the diagnosis of amyloidosis is ?

a. Colonoscopy

b. Rectal biopsy

c. Upper GI endoscopy

d. CT scan

3.] Bone marrow in AL amyloidosis shows ?

a. Bone marrow plasmacytosis

b. Granulomatous reaction

c. Fibrosis

d. Giant cell formation

4.] Which one the following stain is specific for amyloid ?

a. Periodic acid Schiff ( PAS )

b. Alizarin red

c. Congo red

d. Von – Kossa

5.] Amyloid is ?

a. Mucopolysaccaride

b. Lipoprotein

c. Glycoprotein

d. Intermediate filament

6.] Most common site of biopsy in amyloidosis ?

a. Liver

b. Spleen

c. Kidney

d. Lung

7.] Serum amyloid associated protein is found in ?

a. Alzheimer’s disease

b. Chronic inflammatory states

c. Chronic renal failure

d. Malignant hypertension

8.] Amyloidosis is most commonly seen in ?

a. Maturity onset DM

b. Type 1 DM

c. Type 2 DM

d. Equally seen with all forms of DM

9.] Cause of death in amyloidosis involving kidney ?

a. Cardiac failure

b. Renal failure

c. Sepsis

d. Liver failure

10.] On Congo – red staining amyloid is seen as ?

a. Dark brown color

b. Blue color

c. Brilliant pink color

d. Khaki color

 

SOLUTIONS :-

1.] (b) AA

2.] (b) Rectal biopsy

3.] (a) Bone marrow plasmacytosis

4.] (c) Congo red

5.] (c) Glycoprotein

6.] (c) Kidney

7.] (b) Chronic inflammatory states

8.] (c) Type 2 DM

9.] (b) Renal failure

10.] (c) Brilliant pink color

 

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REFERENCES :-

1.] Textbook of Pathology By Harsh Mohan; 7th edition; Page no.66 – 77.

2.] Robbin’s Basic Pathology; 5th edition; Page no.231 – 238.

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