Path: disease of blood vessels Flashcards Preview

CVS > Path: disease of blood vessels > Flashcards

Flashcards in Path: disease of blood vessels Deck (40):

Congenital anomalies of blood vessels (benign) 1

-Vascular malformations: birthmarks, due to errors in vascular morphogenesis (benign), has normal cell turnover/growth
-Capillary malformations (nevus simplex and flammeus): malformed dilated blood vessels that are not cancerous and appear as blotches of red or purple (will involute)


Congenital anomalies of blood vessels (benign) 2

-Nevus simplex (blanching): single or multiple blanchable pink-red patches, often found on nape of neck and btwn eyes, usually spontaneously disappear
-Nevus flammeus (port-wine stain): usually on head/neck and likes the V1-V3 dermatomes, change from pink to red to purple and do not disappear


Hemangiomas of infancy

-Bening vascular tumors (non blanching) often on head and neck, looks like red macule that slowly grows over first few months
-2 phases: first is rapid growth and proliferation over 6-12 mo, then slow involution over several years
-Most follow benign course


Venous and arteriovenous malformations

-Venous: soft compressible blue masses, pain common and may have venous stasis leading to localized coagulopathy
-AVM: usually in brain but can also be in head and neck, may hemorrhage or lead to seizures, headache or progressive neurological deficit
-AVMs may decrease TPR and lead to increase in SV and CO, which can cause cardiac failure


Coarctation of aorta (CoA)

-Congenital narrowing of the aorta arising at level of ductus arteriosus/ligamentum arteriosum
-May occur w/ other defects, such as bicuspid Ao valve, Ao stenosis and IV septal defect, berry aneurysms
-2 types: preductal (infantile) which is more severe (lethal) and less common, and postductal (adult) which is more common and less severe


Pre vs postductal coarctations

-Preductal: narrowing of aorta is proximal to ductus arteriosus, which remains patent and results in cyanosis of lower half of the body and eventual heart failure
-Postductal: clinically it depends on the degree of narrowing, but the narrowing is distal to the ligamentum arteriosus
-Typically there is hypertension in upper body and weak pulses/hypotension in lower extremities w/ potential ischemia
-Often will see intercostal arteries enlarged which causes erosion of the undersurface of the ribs which causes characteristic x ray findings (rib notching)


Hereditary hemorrhagic telangiectasia

-Manifests later in life, no Sx at birth
-AVM apparent after birth (incl plum AVM), recurrent epitaxis, mucocutaneous and GI telangiectasias develop progressively w/ age
-Fe deficiency anemia due to hemorrhage
-Dx requirements: epitaxis, telangiectasias, visceral lesions, family Hx
-Commonly associated w/ 2 genes



-3 forms of artery hardening
-Monckeberg arteriosclerosis



-Presence of fatty plaque within intima of elastic and muscular arteries
-Can lead to major consequences: aneurysm, MI, CVA, gangrene, etc
-Prominent around site of branching b/c of turbulence
-Fibrous cap overlying soft, necrotic lipid core containing foam cells, lymphocytes, cholesterol crystals
-May have neovascularization of the plaque from the vasa vasorum


Complicated atherosclerotic lesions

-Focal erosions or rupture of fibrous cap leading to thrombus formation
-Aneurysmal dilation


Formation of atheromas

-Fatty streaks are precursor, leading to a chronic inflammation of the intima and fibroproliferative process
-Endothelial injury is trigger of inflammation (HTN, hyperlipidemia, DM, smoking)
-Injury is followed by endothelial dysfxn, lowered expression of vasodilatory molecules, increased constriction and pro-inflammatory markers unregulated
-LDL is oxidized and consumed by macs and VSMCs to become foam cells, leading to T cell activation
-VSMCs migrate to surface of intima and contribute to formation of fibrous cap


Monckenberg Arteriosclerosis

-Ring-like calcifications within the media of muscular arteries
-Ca not associated w/ any inflammation, intima and adventitia unaffected
-Does not narrow the lumen



-Arteriosclerotic changes in small arteries and arterioles
-Associated w/ HTN and DM



-Pathologic dilation of a blood vessel or wall of the heart
-True vs false lumen: true is where normal blood flow is, false is where stagnant blood flow, often clot, is due to rupture of the intimal and medial layers and blood collecting in adventitia
-Different formations of aneurysms: saccular (spherical dilation), berry (CoW), fusiform (symmetrical dilation of full vessel circumference)


Subtypes of aneurysms

-Abdominal Ao aneurysms (AAA): most common, usually due to advanced atherosclerosis
-Other causes of AAA: medial degeneration
-AAAs are usually below renal arteries and above ilial bifurcation
-Mycotic aneurysms: infection of vessel wall, usually due to septic embolus from infective endocarditis
-Syphilitic aneurysm: during tertiary syphilis, lesions usually in ascending/thoracic aorta
-Causes arteritis of vasa vasorum leading to ischemia, necrosis of media, dilation of aortic root, and secondary aortic valve incompetence


Aortic dissection (AD)

-Tear in intima and media leads to blood entering a new false lumen
-Blood pools btwn outer 1/3rd and inner 2/3rds of media, usually in descending (type B) or ascending Ao, or both (type A)
-Rupture of Ao leads to massive hemorrhage
-Blood in false lumen can reenter the true lumen thru a second distal tear (double-barreled)
-Ao regurg, MI, cardiac tamponade (blood in pericardium) and other infarctions are common complications


Risk factors of AD

-HTN, weakness in Ao wall, male sex
-Atherosclerosis, CT disease (marfan), old age, underlying aneurysm
-Trauma, medical procedures
-Will often see medial degeneration w/ patchy loss of elastic fibers and SMCs replaced by ECM


Clinical presentation of AD

-Severe, abrupt retrosternal and/or inter scapular pain, described as tearing or ripping or sharp
-Pain may migrate down the back as the dissection progresses
-May see HTN, pulse deficit, SOB, cardiac murmur, or focal neuro deficit (due to infarction)



->140/90, 95% of cases are unknown etiology (essential HTN) and 5% are secondary to something
-Benign HTN: longstanding modest and stable HTN w/o Sx
-Malignant HTN: severe HTN with acute end-organ damage (defined by presence of organ damage and not a certain BP level)


Morphologic changes of large arteries in HTN

-Large arteries: loss of elastic lamina arrangement w/ fiber thinning, intimal hyperplasia, medial SMC hypertrophy and increased ECM deposition
-Changes result in arteriosclerosis, incl increased stiffness, decreased distensibility and subsequent increase in BP
-Also result in Ao dilation


Morphologic changes of small arteries in HTN

-Vessels undergo autoregulation, in which VSMCs respond by vasoconstriction to protect capillary perfusion
-Vessels undergo eutrophic or hypertrophic remodeling
-Eutrophic: better form of remodeling, results in smaller diameter but no change in medial cross-sectional area (no growth in wall mass, but vessel shrinks)
-Hypertrophic: worse outcome, due to increase in vessel wall thickness (mostly media) due to hypertrophy and hyperplasia of VSMCs (associated w/ more severe HTN)


Other affects of HTN

-Endothelial dysfunction and inflammation (from damage) resulting in plaque formation
-Organ damage (mostly heart, brain and kidney) due to narrowing of small blood vessels causing ischemia


Benign nephrosclerosis (BN) 1

-Associated w/ benign HTN, can see hyaline thickening of vessel walls (due to deposition of plasma proteins and increased basement membrane), known as hyaline arteriosclerosis
-Can see amorphous, glassy pink deposition within the vessels


Benign nephrosclerosis (BN) 2

-Luminal narrowing and decreased perfusion results in chronic ischemia
-Leads to scarring (chronic process) from tissue damage: patchy tubular atrophy and interstitial fibrosis w/ potential sclerosis (usually very little inflammation
-Grossly, there is lumpy bumpy appearance of kidneys
-Clinically, there is mild renal dysfxn but a higher risk of progression to renal failure


Malignant nephrosclerosis (MN) 1

-Acute process (associated w/ malignant HTN): non-uniform fibrinoid necrosis, secondary to EC injury
-Inflammation minimal and acute ishcemia/infarction due to diffuse thrombosis
-Healing phase: marked intimal fibrosis (onion-skinning) characterized by concentrically oriented SMCs and ECM deposition


Malignant nephrosclerosis (MN) 2

-Leads to severe luminal narrowing (hyper plastic arteriosclerosis)
-Grossly, diffuse punctate cortical petechial hemorrhages (flea-bitten appearance)
-Renin levels increase and exacerbate the malignant HTN (often seen in secondary HTN)



-Inflammation and damage to vessel walls either due to immune-mediate (most common) or infectious causes


Large vessel vasculitis: giant cell arteritis (GCA)

-AKA temporal arteritis, is same as takayasu arteritis but GCA is in pts >50 and TA is in pts 70, mainly affects arteries in head (temporal)
-Focal segmental granulomatous inflammation with nodular thickening due to intimal hyperplasia (reduction of lumen)
-Can see multinucleate giant cells and lymphocytic infiltrates, fragmentation of IEL, intimal thickening, medial fibrosis, organization, and thrombosis
-Clinically there are headaches, temporal pain, swelling/tenderness, ocular Sx in 50% of pts


Large vessel vasculitis: Takayasu arteritis (TA)

-Essentially GCA in pts <50, histologically similar to GCA
-Clinically can affect any organ system
-Primarily a granulomatous vasculitis of the Ao


Medium vessel vasculitis: polyarteritis nodosa (PAN) 1

-Associated w/ hep B virus, often in young adults
-Localized necrotizing inflammation, favoring bifuctations
-Most commonly affects renal and visceral vessels, but pulmonary circulation is spared
-Lesions can be in many places and at different stages (chronic or acute), can cause mini aneurysm formation


Medium vessel vasculitis: polyarteritis nodosa (PAN) 2

-Acute lesions: perivascular infiltration of PMNs w/ fibrinoid necrosis, may be able to palpate superficial nodules of aneurysms
-Usually cause intravascular thrombosis and infarction
-Chronic lesions: infiltration of mononuclear cells w/ transmural scarring and thickening which may obliterate lumen
-Highly variable Sx depending on organ system involvement, unRx is bad prognosis


Small vessel vasculitis: microscopic polyangiitis

-Vasculitis of capillaries, small arterioles and venules
-Can see segmental necrosis of tunica media, but no granulomas (inflammation dominated by PMNs)
-Lesions are all at same time points developmentally
-Lesions can be found in small vessels of lungs


Small vessel vasculitis: henoch-schonlein puprpura (HSP) 1

-Mostly in children (around 6), multi systemic disease mostly affecting skin, joints, GI, and kidneys
-Due to immune complex deposition (IgA) in vessel walls
-Various inciting agents including URT infections
-Histologically similar to microscopic polyangiitis
-Palpable purport required for Dx, size can vary from pinpoint petechiae to coalescent ecchymoses, usually concentrated on butt and LE


Small vessel vasculitis: henoch-schonlein puprpura (HSP) 2

-Arthralgias/arthritis second most common Sx, often involving knees, ankles, or feet
-GI Sx, ab pain, vomiting, occult or frank bleeding, nephritis w/ or w/o proteinuria/hematuria possible
-Serum IgA levels elevated in most pts
-Can cause thrombosis but most worried about narrowing of lumen and ischemia due to inflammation/IgA deposition


Thromboangiitis obliterans

-Almost exclusively in heavy tobacco smokers/users
-Characterized by focal acute and chronic inflammation, thrombosis of medium and small arteries (particularly tibial and radial arteries)
-Often see ischemia and gangrene in extremities


Antineutrophil cytoplasmic antibodies (ANCA)

-Often associated w/ microscopic polyangiitis and other vasculitis syndromes


Raynaud phenomenon (RP) 1

-Refers to arterial vasospasm and ischemic changes of the digits in the hands and feet that occur primarily following cold exposure and emotional stress
-Primary RP means Sx occur idiopathically, secondary RP from an underlying source (primary is less severe), both are more prevalent in women
-See sequential color change of the digits (white-blue-red)


Raynaud phenomenon (RP) 2

-In 1o there's normal histology in early stages, w/ intimal thickening and thrombus formation in advanced stages
-In 2o theres vascular endothelial damage and corresponding inflammation, w/ more common thrombus formation and hemorrhage
-Pts have sensation of pain, numbness upon ischemia, and pain/throbbing upon reperfusion


AIDS associated kaposi sarcoma (KS)

-Besides AIDS, long-term immunosuppression can also cause KS
-Most cases associated w/ HHV8
-Gross: multiple red-purple skin plaques or nodules, usually in distal LE
-Histologically: irregular endothelial cell-lined vascular spaces w/ interspersed lymphocytes, plasma cells, and macrophages



-Rare aggressive neoplasm from either vascular or lymphatic endothelium
-Most often occurs in head and neck, particularly in elderly men
-Associated w/ chemical carcinogens