vessel pathology Flashcards
large aa
aka elastic
aorta, its large brr, and pulmonary aa
medium sized aa
aka muscular
other brr of aorta
small aa
< 2mm in diameter
vasa vasorum
small aa that feed medium and large aa
in outer 1/2-2/3 medulla
arterioles
20-100um
w/in substance of tissues and organs
veins
venous valves in extremities large diameter large lumen 2/3 of all the blood is in vv thin less well organized walls
post capillary venules
site of leukocyte exudation and vascular leakage
capillaries
approximately he diameter of red blood cell or larger
endothelial cell lining (no media)
surrounded by pericytes
rapid exchange of diffusible substances
lymphatics
endothelial cell lining (no media)
valves in larger lymph vessels
endothelial cells
contain weibel palade bodies
junctions normally impermeable to large molecules
weibel-palade bodies
membrane bound storage organelles that contain vWF
anticoagulant, antithrombotic, fribrinolytics from endothelium
prostacyclin
thrombomodulin
heparin-like molecules
plasminoen activator
prothrombotics from endothelium
vWF
TF
Plasminogen activator inhibitor
modulators of blood flow from endothelium
vasoconstrictors (endothelin, ace)
vasodilators (NO, prostacyclin)
regulators of inflammation and immunity from endothelium
IL1, IL6, chemokines
adhesion molecules( VCAM-1, ICAM, E-selectin, P-selectin)
histocompatilibility Ags
regulators of cell growth from endothelium
stimulators (PDGF, CSF, FGF)
inhibitors (heparin, TGFbeta)
other functions of endothelium
maintenance of permeability
oxidation of LDL
vascular smooth mm
vasoconstriction and dilation
GFs and cytokines
migrate to intima and proliferate following injury
synthesize collagen, elastin, and proteoglycans
can have phagocytic activity
berry aneurysms
aka congenital or berry
2% of autopsies
most in circle of willis
saccular type aneurysm in aa
arteriovenous fistulas
rare abnormal communications btwn aa and vv
most congenital, some produces
short-circuit blood and caue heart to pump additional volume
accelerated HTN
end organ damage
malignant HTN
accelerated plus papilledema
renal causes of HTN
acute glomerulonephritis chronic renal disease polycystic disease renal aa stenosis renal vasculitis renin-producing tumor
endocrine causes of HTN
cushings primary aldosteronism congenital adrenal hyperpalsia licorice exogenous hormones pheochromocytoma acromegaly hypo/hyper thyroidism pregnancy
CV causes of HTN
coarctation of aorta polyarteritis nodosa increased intravascular volume increased CO rigidity of aorta
neurologic causes of HTN
phychogenic
increased intracranial pressure
sleep apnea
acute stress, inducing surgery
liddle syndrome
moderatly severe salt sensitive HTN due to increased distal tubular reabsorption of NA due to over reaction to aldosterone stimulation
3 patterns of arteriosceloris
monckeberg medial cacific scleriosis
arterioloscerosis
atherosclerosis
monckeberg medial calcific sclerosis
in mm aa w/no vessel lumen narrowing and ossify
arteriolosclerosis
in small aa and arterioles
hyaline arterolosclerosis or hyperplastic arterioloscerlosis
hyaline arteriolosclerosis
protein deposition (hyalinized) seen in aging, DM, benign nephrosclerosis, HTN
hyperplastic arterilosclerosis
cell death
onion skinning
+/- necrotizing arteriolitis
seen in malignant HTN
non modifiable risk factors for atherosclerosis
genetics
family Hx
increasing age
male gender
modifiable risk factors for atherosclerosis
hyperlipidemia HTN cigarette smoking DM inflammation
metabolic syndrome
obesity, dyslipedemia, HTn, and insulin resistance
can also have hypercoagulability and inflammatory state
class II LDLR mutations
most common
receptor protein transport form ER to golgi impaired due to abnormal protein folding
lipoprotein a
associated w/higher risk of coronary and cerebrovascular disease
elevated plasminogen activator inhibitor 1
strong predictor of risk for major atherosclerotic events
acute phase reactant
synthesized primarily by the liver
opsonizes bacteria and activating C’
predicts the risk of MI, stroke, peripheral arterial disease, and sudden cardiac death
lowering CRP itself does not decrease risk
smoking cessation, weight loss, statins, and exercise all reduce CRP
infections which may contribute to atherosclerosis
chlamydia pneumoniae, herpesvirus, and cytomegalovirus
COX 1 inhibitors
aspirin
preferentially inhibits COX1 at low doses
blocks thromboxane and increases prostacyclins
COX2 inhibitors
NSAIDS
blocks prostacyclins and increases thromboxanes
abdominal aneurysms
atherosclerosis
M>F, smokers, 50+
thoracic aneurysms
HTN
other causes of aneurysms
marfans, loeys-dietz, ehlers-danos, scurvey, trauma, congenital defects, syphiils and vasculitides
mycotic aneuysms
infections
from embolization of spetic embolus (infective endocarditis)
from extension of adjacent suppurative process
from circulating organisms infecting arterial wall
true aneuysms
bounded by arterial wall component or myocardium
sacular aneurysm
appears rounded
fusiform aneurysm
ivolvoes long segment of a and is not rounded
false aneurysm
aka pedudoaneurysm
hematoma secondary to trasmural rupture
inflamatory abdominal aortic aneurysm
5-10%
rich in lymphocytes, plasma cells, and macrophages
often giant cells
cause uncertain, usually occur at younger age
mycotic abdominal aortic aneurysms
atherosclerotic AAA that have become infected
salmonella gastroenteritis
risk of rupture
6cm 25%
greater then 5cm surgically repaired
obliterative endarteritis
tertiary stage of syphilis (leus) involces vasa vasorum (arterioles) of thoracic aorta
syphilitic aortits
obliterative endarteritis of vasa -> ischemic injury of media -> loss of medial elastic fibuers and mm cells
can lead to aneurysmal dilation -> aortic valve imcompetence
tree-barking appearance
aortic dissection
> 90% men
40-60% w/HTN
arteriolosclerosis -> smooth m loss
in younger people may be CT disorder (marfans, ehlers-danlos, scurvey)
can be iatrogenic
rarely associated w/pregnancy
occasionally secondary to vasa vasorum rupture
Debakey I
begins in ascending aorta and extends into descending aorta
Debakey II
isolated to ascending aorta
Debakey III
isolated to descending aorta
Type A
debakey I and II
begins in ascending aorta
Type B
debakey III
isolated to descending aorta
Marfans Syndrome
loss of fnx mutation of fibrillin
FBN1 gene
autosomal dominant, 25% sporadic
due to decreased fibrillin-1 there is increased TGFbeta -> growth
loeys-dietz syndrome
type II marfans
due to mutations in TGFbetaR
infectious vasculitis
bacterial- nisseria rickettsial- RMSF Spirochetal- syphilis fungal- aspergillosis, mucormcoses viral- herpes-zoster, varicella
IC mediated vasculitis
infection (hep B,C) Henoch-schonlien purpura lupus RA polyarteritis nodosa drug induced hypersensitivity cyroglomulinemia serum sickness
ANCA mediated vasculitis
wegener granulomatosis
microscopic polyangitits
churg strauss syndrome
direct AB mediated vasculitis
goodpastures
kawasaki disease
cell meidated vasculitis
oran allograft rejection
inflammatory bowel disease
paraneoplastic vasculits
unknown vasculitis
giant cell
takayasu arteritis
non-specific symptoms of vasculitis
fever weight loss myalgia arthralgia malaise fatigue thrombosis ischemia nerve pain (depends of cause)
large vessel vasculits
giant cell (temporal) arteritis takayasu arteritis
medium vessel vasculitis
polyarteritis nodosa
kawasaki disease
small vessel vasculitis
wegners
churg-strauss syndrome
microscopic polyangitis
MPO-ANCA
aka p-ANca
antineutrophil cytoplasmic Abs directed against myeloperoxidase
PR3-ANCA
aka c-ANCA
antineutrophil cytoplasmic Abs
against proteinase 3
giant cell arteritis
aka temporal
most comon
usually >50
ophtalmic aa involvement -> diploplia and permanent blindness
may cause giant cell aoritis and thoraci aortic aneurysm
can involve coronary aa
2/3 have anti-endothelial or anti-smooth m Abs
takayasu arteritis
granulomatous inflammation of aorta dn brr
less then 50, females < 40
marked weakening of pulses -> aka pulseless disease) and reduced BP of UL
aa involved w/takayasu arteritis
ocular pulmonary coronary renal aortic valve insufficiency
polyarteritis nodosa
necrotizing inflammation in small to medium aa, arterioles, capillaries, or venules usually young adults renal arterial involvement COD no glomerulonephrtis 30% have hep B Ag/Ab complexes treat w/steroids and cyclophosphamide
kawasaki disease
large, medium, or small aa
usually self limitee in cheildren
anti-endothelial cell and smooth m autoAbs
coronary aa often infvolved
amin cause of aquired pediatric heart disease in north american and epidemic in Japan
kawasaki disease is associated with
mucocutaneous lymph node syndrome
acute self-limiting disease w/fever, conjuctival and oral erythema, strawberry tongue, and erosion, edema of hands and feet, erythema of palms and soles, desquamative skin rash and enlargement of cervical lymph nodes
microscopic polyangitis
aka leukocytoclastic vascultis
necrotizing vasculitis w/few or no immune deposits (pauci immune)
p-ANCA
presentation of microscopic polyangitis
palpable purpura of skin
necrotizing glomerulonephritis
hemoptysis, arthralgia, abdominal pain or bleeding, hematuria, proteinuria, hemorrhage, and m pain or weakness
churg strauss syndrome
aka allergic granulomatosis and angiitis Eos!!!! necrotizing vasculitis involves respiratory tract strong association w/allergic rhinitis, bronchial asthma, lung infiltrates blood eosinophilia p-ANCA
presentation of churg strauss syndrome
Eos!!
palpable purpura
coronary arteritis and eosinophilic myocarditis
severe renal disease infrequent
bahcet disease
neutrophillic vasculits of small-medium aa
triad: recurrent oral aphthous ulcers, genital ulcers, uveitis
can also have GI, pulmonary, and neuro symptoms
can rupture aneurysms
associated w/HLA-B51
can have positive pathergy test- pin prick ulcerates
Wegeners
aka granulomatosis w/polyangitis
triad- acute necortizing granulomas of upper and lower respiratory tract
nectozings or granulomatous vasculitis affecting small-med vessels
renal disease in form of focal necrotizing, often crescentric glomeruloneprhtis w/hematuria and proteinuria +/- renal failure
c-ANCA
Clinical presentation of wegeners
M>F, about 40 persistent pneumonitis, bilateral nodular and cavitary inflitraes chronic sinusitis in 90% mucosal ulcerations of nasopharynx renal disease skin rashes mm pain articular involvement mononeuritis or polyneuritis fever responds to chemo, steroids, anti TNF
Thromboangiitis olterans
aka beurgers disese
semental thrombosin, acute and chronic inflammation of medium sized an small aa (tibial, radial) extending into vv and nn of extremities
buerger disease presentation
cigarette smokers <35 chronic ulceration, toes, feet, fingers, and gangrene secondary raynauds exercise induced instep claudication severe pain, even at rest stop smoking
infectious vasculitis
sometimes from direct invasion of bacteria or fungi (aspergillus and mucormycosis)
can frequently accompany bacteria pneumonia
can occur adjacent to caseous TB abscesses
can occur in superficial cerebral menigitis
may be secondary to septic emboli
may result in mycotic aneurysms or induce thrombotic infarction
frostnip
vasoconstriction fingers, nose, toes-> discoloration
chilblain
aka perniones/perniosis
due to non-freezing temps and damp conditions
chronic, recurrent vasculitis w/red raised lesions
immersion foot
aka trench fot
feet have been wet, but not freezing
may not heal
chronic pain, edema, and blotchy discoloration
often produces superficial, moist, liquefactive gangrene
frostbite
sudden sharp drops in temp
vasoconstriction and increased viscosity of blood
hyperemia and edema
large clear blisters, vesicles filled w/hemorrhagic fluid
dry gangrene
primary raynauds
cold or emotion induced vasoconstirction
late in course inimal thickening can occur
median age 14
distal- proximal: blue-white-red
secondary raynauds
SLE, systemic sclerosis, atherosclerosis, buergers
phelbosclerosis
elastic tissue degredation and spotty calcifications w/in media
can occur w/varicose vv
superior venal caval syndrome
usually neoplasm compresses or invades w/cyanosis and dilation of vv of head, neck, and arms
if pulmonary vessels also compressed causes respiratory distress
inferior venal caval syndrome
neoplam (renal cell, hepatic cell, and adrenal Cx carcinoma) or a thrombus propagates upward
leg edema, lower abdominal distention, superficial collateral vv distention
if renal vv involved massive proteinuria
lymphangitis
infections involving lymphatics w/red streaks
group A beta hemolytic strep
in severe cases produces cellulits or focal abscesses
acute lymphadenitis
red streaks w/painful enlarged regional lymph nodes
obstructive (secondary) lymphedema
dilation of lymphatics w/increased interstitial fluid
caused by:
-malignant tumors obstructing lymphatic channels or lymph nodes
-removal of regional nodes
postirradiation fibrosis
filariasis
postinflammatory thrombosis and scarring
chronic edema can lead to thickened skin- brawny induration or peau d’orange
primary lymphedema
- isolated congenital defect
- familial Milroy disease due to lymphatic agenesis or hypoplasia
- lympedema praexox- age 10-25, usually female, unknown cause, edema begins in feet and accumulates
chylous ascites, chylothorax, chylopericardium
caused by rupture of obstructed lymphatics into peritoneum, pleural cavity, or pericardium
benign neoplasms
hemangiomas (capillary, cavernous, pyogenic granuloma)
lymphangioma (simple/capillary, cavernous)
glomus tumors
non-neoplasm benign issues
vascular ectasias (nevus flammeus, spider telangiectasia, hereditary hemorrhagic telangiectasis) reactive vascular proliferations (bacillary angiomatosis)
intermediate grade- malignant neoplasms
kaposi sarcoma
hemagioednothelioma
malignant neoplasms
agniosarcoma
hemagiopericytoma
hemangiomas
angiomatosis- large segment of body
malignant transformations are rare
7% of all benign tumors in infancy and childhood
common in skin and liver
capillary hemangiomas
- most common vascular tumor, usually skin, subQ, and mucous membranes, but can be in any organ w/a vascular supply
- lumina may be thrombosed, ruputure of vessels can casues scarring w/hemosiderin pigments
juvenile hemangioma
strawberry type
capillary hemagiomas of skin in newborns
grows rapidly in first few months and regresses by age 7 in 75-90%
cavernous hemangioma
larger, less circumscribed, and more frequently involves deep structures
may be locally destructive
no tendency to regress
intravascular thrombosis w/associated dystrophic calcification
hemangiomas of the brain threatening
hemagioblastomas
+/- cavernous hemagiomas in von hippel lindau disease
angiomatous lesions cerebellum, brainstem, and eye
cystic neoplasms in pancreas and liver
other visceral neoplasms
pyogenic granuloma
aka lobular capillary hemagioma polypoid capillary hemagioma rapidly growing red nodule skin or gingival or oral mucosa bleeds easily and often ulcerated 1/3 post trauma edema and inflammatory infiltrate
granuloma gravidarum
pyogenic granuloma that occurs in gingiva of 1% of pregnant women
regresses postpartum
lymphangioma circumscriptum
aka capillary/simple lymphangioma
small lymphatic channels
tend to occur subQ in head and neck and axilla
absence of luminal lymphangioma
cavernous lymphangioma
aka cystic hygroma
in children in neck or axilla, and rarely retroperitoneal
massively dialted cystic lymphatic spaces
occur in turners syndrome
genetic testing asap
glomus tumors
aka glomangioma benign PAINFUL modified cells of glomus body (anteriovenous anasomosis) mostly in distal digits small, elevated, rounded, red-blue, firm nodules
telangiectasia
congenital anomaly or acquired permanent exaggeration of preformed vessels
nevus flammeus
birthmark
most common form of sctasia
port wine stains- usually don’t fade)
sturge-weber syndrome
sturge-weber syndrome
aka encephalotrigeminal angiomatosis
uncommon congenital disorder
venous angiomatous masses in leptomengies
ispilateral port-wine stain nevi w/trigeminal n distribution
often associated w/metal retardation, seizures, hemiplegia, and radiopacities in skull
spider telangiectasia
dialted subQ aa or arterioles about a central core that blanches when pressure applied to center
usually face, neck, or upper chest
most frequent in pregnant women and cirrhosis of liver due to increased E2
hereditaty hemorrhagic telangiectasia
aka osler-weber-rendu disease
congenital malformations consisting of dilated capillaries and vv
present at birth and distributed widely over skin and mucous membranes of oral cavity, lips, respiratory, GI, and GU tracts
autosomal dominant
TGFbeta signaling mutations
bacillary angiomatosis
gram neg bacilli of bartonella family first seen in aids skin, bone, brain domestic cat is reservoir and cat flea vector -> cat scratch disease b quintana- trench fever HIF1alpha
bacillary peliosis
closely related to bacillary angiomatosis vascular lesion
liver and spleen
kaposi sarcoma
HHV-8/KSHV 95% of kaposi lesions are infected w/KSHV infects endothelial cells viral induced G protein induces VEGF immunosupressed and AIDS patients most are asymptomatic
kaposi stages
patch stage
plaque stage
nodular stage
patch stage
pink to purple solitary or multiple macules
dilated angulated blood vessels and chronic inflammatory cells
plaqe stage
larger, violaceous dermal jagged vascular channels, lined by plump spindle cells w/occasional mitoses w/RBC extravasation
nodular stage
raided nodular lesions
often accompanied by involvement of lymph nodes and viscer
sheets of spindle cells w/mitotic figures and slit like spaces w/rows of red cells
types of kaposi sarcoma
chronic/classic/european
lymphadenopathic/african/endemic
transplant/immunosupressed associated
AIDS- associated
chronic KS presentation
-older men of eastern european (ashkenazi jews) or -Mediterranean descent
multiple red-purple skin plaques or nodules, primarily on the arms or legs spreading proximal -viscera or mucosa becomes involved in 10% of patients
chronic KS treatment
resection of primary lesions and recurrences
radiation
chemotherapy for extensive or disseminated disease
lymphadenopathic KS
young bantu children of south africa (same region as burkitt lyphoma)
extreemly aggresive 100% mortality in 3 years
localized or generalized lymphadenopathy
skin lesions sparse
lymphadenopahtic KS treatment
chemo or radiation
transplant associated KS
months-years post transplant w/high doses of immunosuppressants aggressive lymph nodes, mucosa, and visceral organs internal involvement usually fatal skin lesions may be absent
transplant KS treatment
withdrawl or reduction of immunosuppressives
chemo, radiation
AIDs associated KS
most common cancer in US aids patients
lesions of have no site of predilection, but involvement of lymph nodes and gut w/wide dissemination tends to occur early
most patiensts eventually succumb to opportunistic infectious complications of AIDs not KS
AIDs associated KS treatment
antiretroviral therapy
angiogenesis inhibitors may be helpful
hemangioendothelioma
intermediate btwn hemangiomas and angiosarcomas
epithelioid hemangioendothelioma
vascular tumor occurring around medium and large vv in soft tissues of adults
inconspicuous defined vascular channels and tumor cells are plump and often cuboidal
most cured by excision but 50% recur
20-30% metastasize
15% die from metastases
angiosarcoma
malignant and vary formo differentiated to anaplastic
more often in older adults
most common in skin, soft tissue, breast, liver
5 yr survival 30%
can be induced by radiation and w/foreign material
CD31, CD34, Factor VIII, vWF
cutaneous angiosarcoma
small often asymptomatic, red nodules
eventually become large, fleshy, pale, gray-white, with central softening, necrosis, and hemorrhage
