1. Most widely used histochemical technique for differentiating amyloids (from other hyaline materials) = Congo Red Stain [MCQ]
2. Congo Red stain under ordinary light = amyloid appears pink / red.
3. Congo Red stain by polarising microscopy = Green Birefrigence (Most Specific)
4. Amyloid deposition = always Extracellular
5. Cause of congo red staining & birefrigence = Cross-?-pleated conformation.
6. In amyloids, fibril proteins constitute = 95%
7. SAA (serum amyloid-associated)
protein which are the precursors of AA fibril are synthesized in = liver
8. A-Beta protein that is found in Alzheimer's dis. is derived by proteolysis of = Amyloid Precursor Protein.
9. Normal function of Transthyretin (TTR) = binds and transports thyroxine and retinol.
10. Senile Systemic Amyloidosis = Normal TTR.
(Mnem : "Old" TuToR)
11. MC form of amyloidosis = Primary
12. Complicated long term hemodialysis (H/O renal failure), carpal tunnel syndrome= A Beta2 m deposition.
13. Beta2 microglobulin is a component of Class l MHC ; absent in Class ll MHC.
14. Plasma cell disorders are asso. with = Primary amyloidosis.
15. Bence-Jones protein = unpaired kappa or lambda light chains.
16. Amyloidogenic potential of any particular light chain is largely determined by its specific amino acid sequence.
17. Multiple osteolytic lesions in multiple myeloma = NOT Paraneoplastic syndrome.
18. Monoclonal gammopathy and M-spike in serum electrophoresis seen in = Multiple Myeloma.
19. MC association of Systemic (formerly k/a Secondary) amyloidosis is = Rheumatoid arthritis [MCQ]
Followed by Ank. Spond., Chron's disease & Ulcerative colitis.
20. Amyloidosis is reported to occur in = approx. 3% of patients with RA.
21. Subcutaneous Heroin abusers show high risk of = generalized AA amyloidosis. Chronic skin infections asso. with “skin-popping” of narcotics leads to amyloidosis.
22. MC solid tumors asso. with Systemic amyloidosis = RCC and Hodgkin lymphoma.
23. Familial Mediterranean fever = autosomal recessive; autoinflammatory; asso. with excess production of IL-1 in response to inflammatory stimuli.
24. Gene for Familial Mediterranean fever codes for which protein = pyrin.
(Mn : easy to remember as IL1, pyrin are all related to fever)
25. Familial Amyloidotic Polyneuropathies = autosomal dominant.
26. Endocrine Amyloid examples:
- Medullary carcinoma of thyroid
- Islet tumors of the pancreas
- Undifferentiated carcinomas of stomach
- in the Islets of Langerhans
in type 2 DM.
27. MC organ involved in senile amyloidosis = Heart (Restrictive cardiomyopathy & arrhythmias).
28. MC organ involved in localized amyloidosis = Kidney
Most severe localized amyloidosis in = Kidney
29. Localized amyloidosis in organ causes enlargement & grey waxy appearance of the organ except Kidney.
Localized amyloidosis in kidney = normal size & color initially; later shrunken. (Beware!!)
30. First site of amyloid deposition in Kidney = Glomeruli.
First site in Liver = Space of Disse.
First in Heart = focal subendocardial accumulations and within the myocardium.
31. Lardaceous spleen = red pulp
Sago / Topioca-like spleen = white pulp.
33. AL amyloid leads to life-threatening bleeding disorder by inactivating = clotting factor X.
34. Ideal site for biopsy in systemic amyloidosis = Abdominal fat pad
But in localised amyloidosis = that organ (obviously!)
35. Without any doubt, MC organ involved in both Primary & Secondary amyloidosis = Kidney.
36. MC cause of death = Cardiac failure.