One liners in Amyloidosis

Amyloidosis : Oneliners
(Source: Dr. Ranjith's Pathology)

1. Most widely used histochemical technique for differentiating amyloids (from other hyaline materials) = Congo Red Stain

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 beta-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
Followed by Ank. Spond., Crohn'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 
*Pheochromocytomas
*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.