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Flashcards in amyloidosis + other Deck (59)
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1
Q

amyloidosis is the

A

abnormal aggregation of protein (or their fragments into β-sheat

2
Q

amyloidosis –> …. (it causes in the cellular level)

A

damage and apoptosis

3
Q

amyloidosis - common types

A
  1. AL (primary) 2. AA (secondary) 3. dialysis-related
  2. heritable 5. age related (senile) systemic
  3. organ specific
4
Q

primary (AL) amyloidosis - mechanism

A

deposition of proteins from Ig Light chains (amyloid Light: AL)

5
Q

primary (AL) amyloidosis is associated with

A

plasma cell dyscrasias (multiple myeloma)

6
Q

primary (AL) amyloidosis often affects …(including, and causes)

A

multiple organs including renal (nephrotic syndrome), cardiac (restrictive cardiomyopathy, arrhythmia), hematologic, (easy bruising, splenomegaly), GI (hepatomegaly), neurologic (neuropathy)

7
Q

secondary (AA) amyloidosis - seen with

A

chronic inflammatory conditions such as RA, IBD, spondyloarthropathy, protracted infection, famlilial Mediterranean fever

8
Q

secondary (AA) amyloidosis - fibrils composed of

A

serum Amyloid A

9
Q

secondary (AA) amyloidosis - affects

A

often multisystem like AL amyloidosis

10
Q

dialysis related amylodisis - fibrils composed of

A

β2-microglobulin

11
Q

dialysis-related amylodisis - seen in

A

patients with end-stage-renal-disease and/or long-term dialysis

12
Q

dialysis-related amylodisis may present as

A

Carpal tunnel syndrome

13
Q

heritable amyloidosis (including….)?

A

heterogenous group of disorders, including familial amyloid polyneuropathies due to transthyretin gene mutation

14
Q

heritable amyloidosis is a heterogenous group of disorders, including (and due to..)

A

familial amyloid polyneuropathies due to transthyretin gene mutation

15
Q

familial amyloid polyneuropathies are due to

A

transthyretin gene mutation

16
Q

age-related (senile) systemic is due to

A

deposition of normal (wild-type) transthyretin in myocardium and other sites

17
Q

age-related (senile) systemic vs primary amyloidosis according to cardiac function

A

cardiac dysfunction in age-related (senile) systemic has a slower progression relative to primary amyloidosis

18
Q

organ specific amyloidosis?

A

amyloid deposition localized to a single organ

19
Q

organ specific amyloidosis - Most important form is

A

Alzheimer disease

20
Q

Alzheimer disease - mechanism of amyloidosis

A

deposition of β-amyloid protein cleaved from amyloid precirsor protein (APP)

21
Q

islet amyloid polypeptid is commonly seen in …. (and is caused by)

A

diabetes mellitus type 2

deposition of amylin in pancreatic islets

22
Q

Amylin is a

A

peptide hormone that is cosecreted with insulin from the pancreatic beta-cell

23
Q

amyloidosis - appearance

A
  • congo red stain
  • congo red stain shows apple green birefringence under polarized light
  • H% eosin
24
Q

β2-microglobulin fibrils - amyloidosis - type?

A

dialysis-related amyloidosis

25
Q

islet amyloid polypeptid?

A

deposition of amylin in pancreatic islets (DM2)

26
Q

Isolated atrial amyloidosis - due to

A

Organ specific amyloidosis –> due to ANP (COMMON IN NORMAL AGING)

27
Q

p53 - product

A

Transcription factor for p21–> blocks G1 to S phase

28
Q

p16 - product / associated tumor

A

Cyclin-dependent kinase inhibitor 2A

melanoma

29
Q

NF1 / NF2- product

A

Ras GTPase activating protein (neurofibromin)

Merlin (schwannomin protein)

30
Q

PTEN - associated tumor

A
  1. breast
  2. prostate
  3. endometrial
31
Q

TSC1 + TSC2 - products

A

Hamartin protein

Tuberin protein

32
Q

VHL product

A

HIF1a inhibitor

33
Q

BRAF - product + tumor

A

serine/threonine kinase

Melanoma, non-Hodgkin lymphoma

34
Q

BCR-ABL - associated tumor

A

CML, ALL

35
Q

dermatomyositis and polymyositis predispose to visceral malignancy particularly

A

genitourinary

36
Q

immunodeficiency predisposes to (neoplasm)

A
  1. lymphoma
  2. melanoma
  3. renal cell carcinoma
  4. SCC of skin
37
Q

neoplasm - HCV causes

neoplasm - HBV causes

A

hepatocellular carcinoma + LYMPHOMA

38
Q

aromatic amines - examples

A
  • benzidine

- 2- naphthylamine

39
Q

carcinogens - arsenic causes

A
  1. angiosarcoma of the liver
  2. lung cancer
  3. SCC of the skin
40
Q

neoplasms that produce PTHrp

A
  1. SCC of the lung
  2. renal cell carcinoma
  3. breast cancer
  4. bladder
  5. ovarian
  6. head and neck
41
Q

tumor markers - CEA

A
  1. colorectal ca
  2. pancreatic ca
  3. gastric ca
  4. breast ca
  5. medullary thyroid ca
42
Q

breast cancer tumor markers

A
  1. CEA
  2. CA 27-29
  3. CA 15-3
43
Q

tumor markers - β-hCG

A
1. hydatidiform moles
2 .Chroriocarcinomas 
3. testicular ca
4. mixed germ line tumor 
5. Dysgerminoma 
6. Large cell Ca of lung
44
Q

tumor markers - alkaline phosphatase

A
  1. metastasis to bone
  2. metastasis to liver
  3. seminoma (placental alkaline phosphatase)
  4. Paget disease of the bone
45
Q

tumor markers - a-fetoprotein

A
  1. hepatocellular ca
  2. hepatoblastoma
  3. yolk sac (endodermal sinus) tumor
  4. mixed germ cell tumor
46
Q

a-fetoprotein in pregnancy

A

high levels –> neural tube and abdominal wall defects

low levels –> Down syndrome/Patatu (13)/Edwards (18)

47
Q

Psammoma bodies are seen in

A
  1. papillary carcinoma of thyroid
  2. serous papillary cistadenocarcinoma of ovary
  3. meningioma
  4. malignant mesothelioma
48
Q

Good syndrome? - as a paranaeoplastic syndrome - MCC

A
  • hypogammaglobulinemia

- Thymoma

49
Q

pure anemia as a paranaeoplastic syndrome - MCC

A

thymoma

50
Q

carcinomas that spread hematogeneously

A
  1. hepatocellular
  2. renal cell
  3. follicular thyroid
  4. choriocarcinoma
51
Q

Paraneoplastic encephalomyelitis? as a paranaeoplastic syndrome - MCC?

A
  • Antibodies against Hu antigens in neurons

- small cell lung ca

52
Q

Paraneoplastic cerebellar degeneration? as a paranaeoplastic syndrome - MCC?

A
  • Antibodies against Hu, Yo and Tr antigens in Purkinje cells
  • small cell lung ca, gynecologic and breast ca, Hodgkin
53
Q

Anti-NMDA receptor encephalitis? as a paranaeoplastic syndrome - MCC?

A

Psychiatric disturbances, ,e,ory deficits, seizures, dyskenisias, autonomic instability, language dysfunction
ovarian teratoma

54
Q

Opsoclonus myoclonus ataxia syndrome as a paranaeoplastic syndrome - MCC?

A

Neuroblastoma (children)

small cell lung ca (adults)

55
Q

ovarian Ca - paraneoplastic

A
  1. Anti-NMDA receptor encephalitis (ovarian teratoma)

2. PTHrp

56
Q

P-glycoprotein - AKA/seen in

A
  • Multidrug resistance protein 1 (MDR1)

- Classically seen in adrenal cell ca, but also can expressed by other cacner cells (eg. colon. liver)

57
Q

1ry tumors that give metastasis to liver (from the MC)

A

Collon&raquo_space; stomach > pancreas

58
Q

1ry tumors that give metastasis to bone (from the MC)

A

prostate,breast > lung,thyroid,kidnry

59
Q

1ry tumors that give metastasis to brain (from the MC)

A

lung > breast > prostate > melanoma > GI