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PATH FINAL > Haematology PATH > Flashcards

Haematology PATH Flashcards

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1
Q

Hodgkin vs Non-Hodgkin lymphoma

A

Hodgkin
- Single group of nodes
- Spread by contiguity
- Mesenteric nodes and Waldeyers ring rarely involved
- Extranodal involvement uncommon
- Reed-Sternberg cells
- CD30 (no CD 20)

NHL
- Multiple group of nodes
- Non-contiguous spread
- Mesenteric nodes and Waldeyers ring involved
- Extranodal invovlement
- No RS cells
- CD20

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2
Q

Hodgkin Lymphoma
- Epi

A

40% of lymphomas.
Bimodal age peak at 25 and 75. Overall slight male predominance.

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3
Q

4 types of Hodgkin lymphoma

A
  1. Nodular sclerosis (70%)
    - common in women
    - excellent prognosis
  2. Mixed cellularity (20%)
    - EBV associated
  3. Lymphocyte-Rich (uncommon)
    - Good prognosis
  4. Lymphocyte poor
    - WORST prognosis
    - EBV positive*
  5. Lymphocyte preDOMINANCE
    - BEST prognosis
    - Popcorn cells
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4
Q

Hodgkin lymphoma risk factor ?

A
  • EBV
  • Immunosuppression
  • Connective tissue disorders
  • RA, SLE, Sarcoid, UC
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5
Q

Morphology of Hodgkin Lymphoma

A
  • RS cells in a background of non-neoplastic inflammatory cells **
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6
Q

Hodgkin lymphoma prognosis

A
  • Curable in most cases*
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7
Q

How does PTH increase serum calcium ?

A
  1. Kidney
    - increase renal absoprtion
    - increase conversion of vitamin D to active form
  2. Increase GI absoprtion
  3. Increase RANK-L expression to increase osteoclast function
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8
Q

Most common cause of hyperparathyroidism

A
  • The most common cause of primary hyperparathyroidism is a sporadic parathyroid adenoma
  • Familial syndromes are a distant second
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9
Q

How to make the diagnosis of Multiple Myeloma

A
  • Monocloncal immunoglobulin
  • Free Light chains
  • Bence-Jones proteins (directly nephrotoxic)
  • Tamm Horsfall proteins
  • Get amyloidosis AL type in kidney*
  • Rouleaux formation (high M chains)

However, 1% are non-secretors with nothing detectable in blood or urine

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10
Q

What’s the major pathological feature of Multiple Myeloma ?

A

Unregulated expression of RANKL, which activates osteoclasts.

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11
Q

Multiple myeloma
- associations

A

Urate neprhopathy

Amyloidosis

POEMS - an atypical form of myeloma – polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes

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12
Q

Follicular NHL features and prognosis

A
  • Indolent but incurable*
  • 40% transforms to DLBCL
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13
Q

Marginal zone lymphoma (NHL) prognosis

A

Can regress if inciting stimulus removed. Hallmark is lymphoepithelial lesion

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14
Q

Burkitt lymphoma association

A
  • EBV, HIV
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15
Q

Burkitt morphology

A

Fish flesh tumorous masses. Also a term/appearance sometimes for sarcomas.

Distribution: 60% nodal vs 40% extranodal (spleen usually)

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16
Q

What cells do you find in Parathroid hyperplasia ?

A

” Chief cell “ Hyperplasia

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17
Q

B-thalassemia or Alpha more common ? Which one more severe usually

A

B-Thalassemia more common and more severe usually

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18
Q

3 alpha gene deletion and 4 alpha gene deletion is called ?

A

3 alpha gene deletion = HbH (clinically resembles B-thalassemia intermedia)

4 alpha gene deletion = Hb Barts (Long term survival now possible with early recognition, intrauterine transfusion, stem cell transplant)

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19
Q

NF-1 or NF-2 more common?

A

NF-1 by far

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20
Q

NF-1 which chromosome mutation and gene product ?

A

Loss of Chr 17 -> decreased “neurofibromin”

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21
Q

Plexiform Neurofibroma complication ?

A
  • A plexiform neurofibroma has a lifetime risk of up to 10% of transformation into a malignant tumour
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22
Q

Main cause of death for NF-1 ?

Study These Flashcards
A

Malignant peripheral nerve sheath tumor

23
Q

NF-2 epidemiology

Study These Flashcards
A
  • Occurs in 1 in 40-50,000.
  • Rarely noticed prior to puberty
  • Mean age of diagnosis ~25 years
24
Q

NF-2 loss of gene on what chromosome

Study These Flashcards
A
  • Loss of NF2 gene = chr22q12.
  • NF2 functions as a tumour suppressor gene (encodes for Merlin protein).
25
NF-2 prognosis
Poor prognosis, mean age of death 36 Resection of CN8 schwannomas with hearing preservation is possible Genetic counselling
26
TS inheritance ?
- Sporadic most common (60%) - Minority familial: AD - can skip generation due to low expressitivity
27
VHL clinical presentation
Visual loss from retinal haemangioblastoma
28
VHL complication
Renal cell carcinoma is a major cause of morbidity and mortality – 70% risk by age 60.
29
GRANULOMATOSIS with POLYANGIITIS (Wegners) - Pathophysiology ? - C-ANCA positive in how many %?
Probably a T-cell mediated hypersensitivity response to normally “innocuous” inhaled microbial or other environmental agents (Robbins). C-ANCA positive in up to 95% of cases.
30
Granulomatosis with Polyangiitis - most common location ?
Lungs > paranasal sinuses > kidneys > joints > eye > skin
31
Indications for AAA surgery
- ascending aorta >5.5 cm - descending aorta >6.5 cm - growth rate >1cm/year - symptomatic patient
32
Mycotic aneurysm cause ?
- Embolisation of septic embolus (usually from infective endocarditis) - Extension of adjacent suppurative process or circulating organisms directly infect the arterial wall.
33
Inflammatory aneurysm shape ?
- Fusiform
34
Adult form Coarctation of Aorta presentation ?
- Presents later, often well into adulthood - Upper extremity hypertension with weak pulses and hypotension in the lower extremities - Manifestations of arterial insufficiency (ie claudication, cold legs). - Murmurs
35
What vessels causes rib notching in Aortic Coarctation ?
- intercostal and internal mammary arteries
36
Aortic coarctation associations
- Bisucpis aortic valve (50%) - VSD, ASD, mitral regurg, berry aneurysms, aortic stenosis - Turners
37
Infantile vs Adult form Aortic coarctation
Infantile - Preductal Adult - Postductal
38
Stanford A more common or B?
- A more common
39
RF of aortic dissection
- HTN (90%) - Aortic Coarctation - Bicuspid aortic valve - Connective tissue disorders - Cardiac catheterisation
40
Morphology seen in Dissection and ANeurysm
- Cystic medial degeneration - Intimal tear into but not beyond the media – the blood filled false lumen is usually in a plane between the middle and outer 1/3 of the media.
41
Do all aortic dissection have intimal tears ?
No, 5-10% dont have obvious intimal tears
42
Are all aortic dissection detectable on Radiology ?
- No
43
Atheroma development pathophysio first step
Endothelial injury (vascular permeability, leukocyte adhesion)
44
Atheroma composition
- Lipid core with fibrous cap - Periphery (neovascularisation).
45
What are Fatty streaks
- Early lesions made of lipid laden foamy macrophages in the intima that only mildly protrude into the lumen - Occur at any age even in kids and not all go on to become atheromatous plaques, although some do
46
T-cell lymphoma high or low grade ?
T cell = all high grade
47
Primary vs Secondary Syphilis features
Primary syphilis: painless chancre with nontender lymphadenopathy Secondary: Papulosquamous, Rash skin lesions Tertiary: gummatous lesions, neurologic or cardiovascular symptoms
48
Extra-adrenal paraganglioma most common site ?
Approximately 85% arise below the diaphragm
49
Paraganglioma more commonly maglignant if ..?
- Extra-adrenal - Related to syndrome
50
Most common parasympathetic paraganglioma ?
- Carotid body paraganglioma
51
Hashimoto labs ?
- Lymphocytic aggregates - Transformed follicular cells (Askanazy/oxyphilic/Hurthle cells) - Anti-thyroglobulin antibodies (70%) - TPO (95%) - TSH (High) - Free T4 (Low)
52
Hashimoto complication
- Non-hodgkin lymphoma (majority) - Papillary thyroid cancer (1.7x increased risk) - Medullary thyroid cancer (2.7x increased risk)
53

PATH FINAL (12 decks)

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