Week 3: CV pathology diseases of blood vessels I & II Flashcards

1
Q

Describe capillary vascular malformations.

A
  • malformed dilated capillaries in skin. Not a neoplasm.
  • usually present at birth, vary in size, grows in proportion to child’s growth
    1. Nevus simplex (macular stain)
  • aka salmon patch,stork bite, angel kiss
  • blanchable pink-red patches
  • common at nape of neck, forehead between eyebrows, eyelids
  • most disappear within 1st year of life
  • may be more intense with crying
    2. Naevus Flammeus
  • “port wine”
  • common in head/neck, dermatomal dist. for facial lesions
  • pink to red macules at birth, persist and darken with age, may become ticketed, irregular and nodular
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2
Q

Describe hemangiomas of Infancy (HOI)

A
  • benign vascular neoplasm
  • 50% present at birth as red macule,papule, 50% appear in first few months of life
  • 2/3 in head/neck
  • Two phases: rapid growth for 6-12 months, then slow involution over years
  • most follow benign course
  • can have visceral involvement
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3
Q

Describe venous malformations.

A
  • superficial or deep
  • pain common, esp with deeper lesions.
  • venous stasis and activation of altered endothelium may cause localized coagulopathy–>phlebothrombosis common
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4
Q

Descrive arteriovenous malformations (AVM).

A
  • abnormal communication between artery and vein that bypasses capillary bed
  • brain is most common site, greatest clinical significance. Can hemorrhage, cause seizure, headache, progressive neurological deficit
  • In large AVMs, can have high output cardiac failure from decreased peripheral resistance and compensatory increase in SV and CO
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5
Q

Describe lymphatic malformations.

A
  • commonly occurs as lymphatic-venous malformations or capillary-lymphatic malformations
  • cervicofacial and axilla region most common
  • can be associated with trisomy 13,18,21 and Turner syndrome
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6
Q

Describe coarctation of the aorta.

A
  • narrowing of the aorta at the level of ductus arteriosis/ligamentum arteriosum
  • Two types:
    1) pre-ductal (infantile): less common and more severe. Narrowin is proximal to ductus arteriosus, heart failure in early life often. patent ductus arteriosus.
    2. )post ductal (adult): less severe. narrowing is distal to ligamentum arteriosum. Hypertension in upper body and weak pulses/hypotension in lower with potential ischemia. Large intercostal arteries as collateral circulation.
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7
Q

Describe hereditary hemorrhagic telangiectasia.

A
  • autosomal dominant vascular disorder that manifests later in life, not congenital, genetically predetermined
  • variety of manifestations
  • diagnostic criteria: epistaxis, telangiectasias, visceral history, family history
  • can have secondary iron deficiency anemia
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8
Q

List in descending order from the most severely affected, the vessels affected by atherosclerosis and their major clinical consequences.

A
  1. abdominal aorta-aneurysm
  2. proximal coronary artery - MI, chronic ischemic heart dz
  3. descending thoracic aorta - aneurysm
  4. popliteal artery -gangrene
  5. internal carotid artery - cerebral infarct
  6. circle of willis vessels- cerebral infarct
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9
Q

What are complicated lesions (atherosclerosis)?

A

-advanced plaques that undergo certain changes which are particularly significant clinically, including: focal erosion, ulceration or rupture of fibrous cap–>thrombosis and micro emboli, hemorrhage, aneurysmal dilation, calcification

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10
Q

Describe the fatty streak in atherosclerosis?

A
  • aggregates of foam cells (LDL in macrophages), T cells, SMCs, aggregated platelets
  • 1st grossly visible lesion in development of atherosclerosis
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11
Q

What is atherothrombosis?

A

-atherosclerotic plaque disruption with superimposed thrombosis (leading cause of mortality in Western world)

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12
Q

Define aneurysm.

A

=pathologic dilution of a segment of a blood vessel or wall of the heart

  • True aneurysm=dilation of all three intact, but often attenuated vessel wall layers
  • false aneurysm=rupture of the intimal and medial layers, with resulting dilation created by extravasation of blood into the adventitia
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13
Q

What are 3 subtypes of aneurysm, based on shape or location or cause.

A
  1. Abdominal aortic aneurysm: most common. mostly caused by advanced atherosclerosis with erosion of IEL and plaque expansion leading to medial destruction. Or caused by connective tissue disease. Saccular or fusiform.
  2. Mycotic aneurysms: infection of vessel wall, mostly caused by septic embolus in infective endocarditis. Also secondary to neighboring bacterial infection.
  3. Syphilitic: tertiary syphilis.
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14
Q

What are 3 different shapes of aneurysms?

A
  • saccular: assymmetric, spherical
  • berry: small intracranial saccular aneurysms. develops over time due to underlying congenital weakness of vessel media
  • fusiform: progressive, symmetrical dilation of complete vessel circumference.
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15
Q

Describe aortic dissection. Risk factors?

A
  • definition: intimal tear allowing blood to enter vessel wall and dissect along laminar planes of aortic media, forming false channel.
  • plane of dissection: outer 1/3 of media–> weakened external wall can lead to rupture
  • common site: ascending aorta above aortic ring, then descending aorta below ligamentum arteriosum
  • risk factors: age>70, hypertension, male, atherosclerosis, connective tissue disease
  • major complications: acute aortic regurg, MI, cardiac tamponade from rupture into pericardium, potential cerebral/renal/mesenteric infarct
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16
Q

How is aortic dissection classified?

A

-Type A: ascending aortic lesion only or ascending and descending aortic lesion
-Type B: descending aortic lesion only
risk of rupture greater in Type A

17
Q

What are histological findings in aortic dissection?

A

-medial degeneration=patchy loss of elastic fibers and SMCS replaced by myxoid (glycoprotein).

18
Q

Define hypertension. How is it classified?

A

-typically defined as systolic pressure>140 and/or diastolic >90mmHg
-95% is primary, idiopathic, essential HTN
-rest is secondary, e.g. renal abnormalities
Classification
-Benign: no symptoms, longstanding mild/moderate
-malignant: aka hypertensive crisis or emergency, severe HTN with acute end organ damage. Bp usually >200/140 mmHg. Rapid onset.

19
Q

Describe the morphological changes due to hypertension in large arteries.

A
  • elastic fiber degeneration from tissue fatigue w/ thinning and fragmentation of fibers. lower damping ability of vessels to compensate for mechanical stress
  • intimal hyperplasia
  • medial SMC hypertrophy and increased ECM deposition
  • vessel dilation—>potential aneurysm/dissection
20
Q

Describe the morphological changes due to hypertension in small arteries/arterioles.

A

-autoregulation: leads to vasoconstriction due to high bp to protect capillary beds. If chronic, leads to remodeling
-Eutrophic inward remodeling: most common, smaller lumen, no change in medial cross sectional area. Essential HTN.
-Hypertrophic inward remodeling: less common. ECM deposition with increase in medial cross sectional area. worse prognosis. assoc with advanced HTN.
VASCULAR CONSEQUENCES
-mechanical stress: loss of structural integrity, leakage across endothelium, fixed luminal narrowing, luminal obliteration
-Organs: heart, brain, kidneys vulnerable. May lead to renal hypotension—>increase renin–>angiotensin–>aldosterone, worsened HTN

21
Q

Describe Benign Nephrosclerosis.

A

-assoc. w/ benign HTN
EARLY
-vasoconstriction by SM contraction. No microscopic changes
LATER
-arteries: myointimal thickening, IEL changes, medial hypertrophy
-arterioles: hyaline thickening of vessel walls (leakage of plasma proteins across injured ECs and deposition of ECM/basement membrane constituents)
-luminal narrowing and decreased perfusion–>chronic ischemia->nephropathy–>patchy scarring
-interstitial fibrosis with little or no inflammation.
-granular appearance of kidney, areas of scarring (contracted) and raised areas of preserved parenchyma
-affects afferent arteries

22
Q

Describe malignant nephrosclerosis.

A

-associated with malignant HTN.
ACUTE phase
-medial fibrinoid necrosis
-eosinophilic lesion secondary to EC injury, minimal inflammation, platelet deposition–>may cause thrombosis, ischemia, infarct
HEALING phase
-onion skinning and intimal fibrosis
-concentric SMCs and ECM deposition, hyperplastic AS
-severe luminal narrowing/obliteration—>ischemic atrophy, capillary rupture
Grossly: flea bitten kidney, petechial hemorrhages

23
Q

What is vasculitis?

A
  • inflammation of vessel walls
  • important to distinguish between infectious and non infectious causes
  • non infectious due to: immune complex deposition, ANCA (anti neutrophill cytoplasmic antibodies), and anti endothelial cell antibodies
24
Q

How is vasculitis classified?

A
  1. Large vessel
    - Giant cell arteritis -granulomas
    - Takayasu aortitis -granulomas
  2. Medium vessel
    - Polyarteritis nodosa (assoc. w/ Hep B) -PMN, fibrinoid necrosis
    - thromboangiitis obliteran (tobacco smoker)-granulomas
  3. Small vessel
    - Henoch-Schonlein Pupura (HSP)
    - Wegener’s -granuloma
    - microscopic polyangiitis -PMN and fibronoid necrosis
    - Churg-Strauss syndrome - eosinophils
25
Q

Describe Giant Cell Arteritis

A
  • most common vasculitides
  • diagnosed in >50 yo (commonly >70), Caucasians
  • affects arteries in head: swollen, red, tender
  • pathology: nodular thickening due to intimal hyperplasia, intimal/medial granulomatous inflammation w/ giant cells, fragmentation of IEL
  • may have thrombosis
  • clinical: throbbing, headache, ocular symptoms common
26
Q

Describe Takayasu Aortitis

A
  • like Giant Cell arteritis except in young people
  • granulomatous vasculitis of aorta or major branches
  • women, asians, under 50 yo
27
Q

Describe polyarteritis nodosa (PAN)

A

-multiple organs involved, spares lungs, medium sized vessels
-rare, in young adults 40-60yo
-immune complex reaction, assoc with Hep B
-Acute lesions: PMNs w/ fibrinoid necrosis, inflammation, microaneurysms w/ potentially palpable superficial nodule
—>thrombosis and infarct
-Chronic lesions: Mononuclear cells, scarring and fibrotic thickening, compromise or obliterate lumen
CLINICAL: depends on organ involvement

28
Q

Describe microscopic polyangiitis.

A
  • vasculitis of capillaries, small arterioles and small venules
  • inflammatory response dominated by PMNs
  • can affect vessels anywhere, most have kidney involvement
  • palpable cutaneous purpura
  • looks like PAN except for small vessels
29
Q

Describe Henoch-Schonlein Purpura.

A
  • most common vasculitis in kids
  • related to IgA immune complex deposition
  • triggers: Upper Resp infection, drugs/allergies, insect bites, immunizations
  • PMN infiltration of small vessels,
  • palpable purpura, mostly butt and lower extremities
30
Q

Describe thromboangiitis obliterans

A
  • heavy tobacco smokers
  • often leads to ischemia and gangrene of the extremities
  • focal acute and chronic inflammation, and thrombosis of medium and small sized arteries
31
Q

Describe Reynaud Phenomenon.

A
  • arterial vasospasm and ischemic changes of digits on the hands and feet following cold or emotional stress
  • paroxysmal discoloration of fingers, toes, tips of nose/ears
  • young, healthy women
  • Primary RP: histologically normal in early stages, may have intimal thickening and thrombus formation in later stages
  • Secondary RP (secondary to disease, e.g. lupus): vascular endothelial damage and inflammation common, thrombus and/or hemorrhage may occur, ulceration and gangrene
32
Q

What is lymphangitis?

A

-bacterial infections may spread into and through lymphatics–>dilated lymphatics filled with PMNs/Macrophages, cellular debris, and bacteria

33
Q

Describe angiosarcoma.

A
  • rare, aggressive neoplasm from vascular or lymphatic endothelium
  • can be anywhere, skin, breast, and liver common
  • liver angiosarcomas associated with carcinogen exposure
34
Q

Describe granuloma pyogenicum

A
  • rapidly growing pedunculated red nodule on the skin, gingival or oral mucosa
  • often follows trauma