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Ch 32 Secondary GN > AMYLOIDOSIS > Flashcards

AMYLOIDOSIS Flashcards

1
Q

What are the antiparallel β-pleated sheet configuration on X-ray diffraction called?

A

AMYLOID FIBRILS

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2
Q

What 2 stains will amyloid fibrils bind?

A
  1. CONGO RED
  2. THIOFAVIN T
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3
Q

What are the 2 types of amyloidosis?

A
  1. AA AMYLOIDOSIS
  2. AL AMYLOIDOSIS
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4
Q

What is the most common type of Amyloidosis?

A

AL AMYLOIDOSIS

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5
Q

What type of amyloidosis where the deposited fibrils are derived from the variable portion of immunoglobulin light chains produced by a clonal population of plasma cells or plasmacytic B cells?

A

AL AMYLOIDOSIS

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6
Q

What type of amyloidosis results from the deposition of serum amyloid A (SAA) protein in chronic inflammatory states?

A

AA AMYLOIDOSIS

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7
Q

In AL amyloid patients with renal disease what type of chains strongly predominate?

A

λ-LIGHT CHAINS

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8
Q

What is the ratio of λ-light chains to κ-light chains in pxs with renal disease?

A

12:1

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9
Q

What is the ratio of λ-light chains to κ-light chains in pxs without renal disease?

A

4:1

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10
Q

What are the most common major organ involved by AL amyloid?

A

KIDNEYS

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11
Q

What is the age and gender predilection in AL amyloidosis?

A

• > 50 years
• MALES

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12
Q

What type of amyloidosis occurs in chronic inflammatory diseases?

A

AA AMYLOIDOSIS

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13
Q

Where is SAA (Serum Amyloid A) produced?

A

LIVER

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14
Q

What type of lipoprotein does SAA (Serum Amyloid A) associates in the circulation?

A

HDL

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15
Q

AA Amyloid is most commonly associated with?

A

RHEUMATOID ARTHRITIS

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16
Q

AA amyloidosis is also seen in these 7 conditions

A
  1. INFLAMMATORY ARTHRITIDES
  2. IBD
  3. FMF (Familial Mediterranean Fever)
  4. QUADRIPLEGICS with chronic UTI & decubitus ulcers
  5. BRONCHIECTASIS
  6. OSTEOMYELITIS (poorly treated)
  7. CHRONIC HEROIN ADDICTS (Injects drugs SQ)
17
Q

AA Amyloid occurs in what population?

A

older addicts with a long history of substance abuse who have exhausted sites of intravenous access and resorted to “skin popping” or “muscling”

18
Q

The diagnosis of amyloid is usually established by?

A

Tissue biopsy of an affected organ

19
Q

What diagnostic imaging allows the noninvasive diagnosis of amyloidosis as well as a quantification of the extent of organ system involvement and assessment of the response to treatment?

A

SAP WHOLE BODY SCINTIGRAPHY

20
Q

What are the LM findings in Amyloidosis?

A

GLOMERULI:
• deposition of amorphous hyaline material that begins in the mesangium and extends into the peripheral capillary walls
• Affected glomeruli appear hypocellular and have a nodular aspect.
DEPOSITS:
• lightly eosinophilic
• weakly PAS positive
• nonargyrophilic (contrast to the findings in diabetic nodular glomerulosclerosis)
GBM
• amyloid deposits form spicular hairlike projections

21
Q

Congo Red staining gives what appearance?

A
  1. ORANGE STAINING REACTION
  2. APPLE-GREEN BIREFRINGENCE UNDER POKARIZED LIGHT
22
Q

What is the IF findings in AL amyloidosis?

A

strong staining with antisera to the pathogenic light chain, usually λ

23
Q

What is the IF finding in AA Amyloidosis?

A

STRONG REACTIVITY TO SAA PROTEIN

24
Q

What are the characteristics of Amyloid fibrils by EM?

A

• nonbranching
• 8-12 nm
• randomly distributed

25
Q

What is the appearance of glomerular capillary wall?

A

• SPICULAR
• COCKSCOMB-LIKE PROJECTIONS ALONG SUBEPITHELIAL AREAS

26
Q

What are the 3 predictors of progression to ESKD in AL amyloidosis?

A
  1. CREATININE AT BASELINE
  2. PROTEINURIA AT BASELINE
  3. HEMATOLOGIC RESPONSE
27
Q

What are the top 2 primary causes of death in Amyloidosis?

A
  1. CARDIAC INVOLVEMENT (Heart Failure/Arryhtmias)
  2. RENAL DISEASE
28
Q

What treatment is given for healthier and younger patients in AL Amyloidosis?

A

ASCT (AUTOLOGOUS STEM CELL TRANSPLANT)

29
Q

What treatment is given for older patients with more morbid associated conditions?

A

chemotherapeutic regimens

30
Q

What are the other 5 medications used together with dexamethasone used in the treatment of amyloidosis?

A
  1. MELPHALAN
  2. LENALIDOMIDE
  3. THALIDOMIDE
  4. BORTEZOMIB
  5. CYCLOPHOSPHAMIDE

Ch 32 Secondary GN (35 decks)

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