vessel pathology Flashcards

1
Q

large aa

A

aka elastic

aorta, its large brr, and pulmonary aa

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2
Q

medium sized aa

A

aka muscular

other brr of aorta

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3
Q

small aa

A

< 2mm in diameter

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4
Q

vasa vasorum

A

small aa that feed medium and large aa

in outer 1/2-2/3 medulla

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5
Q

arterioles

A

20-100um

w/in substance of tissues and organs

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6
Q

veins

A
venous valves in extremities
large diameter 
large lumen
2/3 of all the blood is in vv
thin less well organized walls
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7
Q

post capillary venules

A

site of leukocyte exudation and vascular leakage

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8
Q

capillaries

A

approximately he diameter of red blood cell or larger
endothelial cell lining (no media)
surrounded by pericytes
rapid exchange of diffusible substances

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9
Q

lymphatics

A

endothelial cell lining (no media)

valves in larger lymph vessels

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10
Q

endothelial cells

A

contain weibel palade bodies

junctions normally impermeable to large molecules

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11
Q

weibel-palade bodies

A

membrane bound storage organelles that contain vWF

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12
Q

anticoagulant, antithrombotic, fribrinolytics from endothelium

A

prostacyclin
thrombomodulin
heparin-like molecules
plasminoen activator

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13
Q

prothrombotics from endothelium

A

vWF
TF
Plasminogen activator inhibitor

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14
Q

modulators of blood flow from endothelium

A

vasoconstrictors (endothelin, ace)

vasodilators (NO, prostacyclin)

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15
Q

regulators of inflammation and immunity from endothelium

A

IL1, IL6, chemokines
adhesion molecules( VCAM-1, ICAM, E-selectin, P-selectin)
histocompatilibility Ags

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16
Q

regulators of cell growth from endothelium

A

stimulators (PDGF, CSF, FGF)

inhibitors (heparin, TGFbeta)

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17
Q

other functions of endothelium

A

maintenance of permeability

oxidation of LDL

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18
Q

vascular smooth mm

A

vasoconstriction and dilation
GFs and cytokines
migrate to intima and proliferate following injury
synthesize collagen, elastin, and proteoglycans
can have phagocytic activity

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19
Q

berry aneurysms

A

aka congenital or berry
2% of autopsies
most in circle of willis
saccular type aneurysm in aa

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20
Q

arteriovenous fistulas

A

rare abnormal communications btwn aa and vv
most congenital, some produces
short-circuit blood and caue heart to pump additional volume

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21
Q

accelerated HTN

A

end organ damage

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22
Q

malignant HTN

A

accelerated plus papilledema

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23
Q

renal causes of HTN

A
acute glomerulonephritis
chronic renal disease
polycystic disease
renal aa stenosis
renal vasculitis
renin-producing tumor
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24
Q

endocrine causes of HTN

A
cushings
primary aldosteronism
congenital adrenal hyperpalsia
licorice
exogenous hormones
pheochromocytoma
acromegaly
hypo/hyper thyroidism
pregnancy
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25
Q

CV causes of HTN

A
coarctation of aorta
polyarteritis nodosa
increased intravascular volume
increased CO
rigidity of aorta
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26
Q

neurologic causes of HTN

A

phychogenic
increased intracranial pressure
sleep apnea
acute stress, inducing surgery

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27
Q

liddle syndrome

A

moderatly severe salt sensitive HTN due to increased distal tubular reabsorption of NA due to over reaction to aldosterone stimulation

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28
Q

3 patterns of arteriosceloris

A

monckeberg medial cacific scleriosis
arterioloscerosis
atherosclerosis

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29
Q

monckeberg medial calcific sclerosis

A

in mm aa w/no vessel lumen narrowing and ossify

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30
Q

arteriolosclerosis

A

in small aa and arterioles

hyaline arterolosclerosis or hyperplastic arterioloscerlosis

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31
Q

hyaline arteriolosclerosis

A
protein deposition (hyalinized)
seen in aging, DM, benign nephrosclerosis, HTN
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32
Q

hyperplastic arterilosclerosis

A

cell death
onion skinning
+/- necrotizing arteriolitis
seen in malignant HTN

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33
Q

non modifiable risk factors for atherosclerosis

A

genetics
family Hx
increasing age
male gender

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34
Q

modifiable risk factors for atherosclerosis

A
hyperlipidemia
HTN
cigarette smoking
DM
inflammation
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35
Q

metabolic syndrome

A

obesity, dyslipedemia, HTn, and insulin resistance

can also have hypercoagulability and inflammatory state

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36
Q

class II LDLR mutations

A

most common

receptor protein transport form ER to golgi impaired due to abnormal protein folding

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37
Q

lipoprotein a

A

associated w/higher risk of coronary and cerebrovascular disease

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38
Q

elevated plasminogen activator inhibitor 1

A

strong predictor of risk for major atherosclerotic events

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39
Q

acute phase reactant

A

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

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40
Q

infections which may contribute to atherosclerosis

A

chlamydia pneumoniae, herpesvirus, and cytomegalovirus

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41
Q

COX 1 inhibitors

A

aspirin
preferentially inhibits COX1 at low doses
blocks thromboxane and increases prostacyclins

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42
Q

COX2 inhibitors

A

NSAIDS

blocks prostacyclins and increases thromboxanes

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43
Q

abdominal aneurysms

A

atherosclerosis

M>F, smokers, 50+

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44
Q

thoracic aneurysms

A

HTN

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45
Q

other causes of aneurysms

A

marfans, loeys-dietz, ehlers-danos, scurvey, trauma, congenital defects, syphiils and vasculitides

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46
Q

mycotic aneuysms

A

infections
from embolization of spetic embolus (infective endocarditis)
from extension of adjacent suppurative process
from circulating organisms infecting arterial wall

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47
Q

true aneuysms

A

bounded by arterial wall component or myocardium

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48
Q

sacular aneurysm

A

appears rounded

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49
Q

fusiform aneurysm

A

ivolvoes long segment of a and is not rounded

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50
Q

false aneurysm

A

aka pedudoaneurysm

hematoma secondary to trasmural rupture

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51
Q

inflamatory abdominal aortic aneurysm

A

5-10%
rich in lymphocytes, plasma cells, and macrophages
often giant cells
cause uncertain, usually occur at younger age

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52
Q

mycotic abdominal aortic aneurysms

A

atherosclerotic AAA that have become infected

salmonella gastroenteritis

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53
Q

risk of rupture

A

6cm 25%

greater then 5cm surgically repaired

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54
Q

obliterative endarteritis

A

tertiary stage of syphilis (leus) involces vasa vasorum (arterioles) of thoracic aorta

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55
Q

syphilitic aortits

A

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

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56
Q

aortic dissection

A

> 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

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57
Q

Debakey I

A

begins in ascending aorta and extends into descending aorta

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58
Q

Debakey II

A

isolated to ascending aorta

59
Q

Debakey III

A

isolated to descending aorta

60
Q

Type A

A

debakey I and II

begins in ascending aorta

61
Q

Type B

A

debakey III

isolated to descending aorta

62
Q

Marfans Syndrome

A

loss of fnx mutation of fibrillin
FBN1 gene
autosomal dominant, 25% sporadic
due to decreased fibrillin-1 there is increased TGFbeta -> growth

63
Q

loeys-dietz syndrome

A

type II marfans

due to mutations in TGFbetaR

64
Q

infectious vasculitis

A
bacterial- nisseria
rickettsial- RMSF
Spirochetal- syphilis
fungal- aspergillosis, mucormcoses
viral- herpes-zoster, varicella
65
Q

IC mediated vasculitis

A
infection (hep B,C)
Henoch-schonlien purpura
lupus
RA
polyarteritis nodosa
drug induced hypersensitivity 
cyroglomulinemia
serum sickness
66
Q

ANCA mediated vasculitis

A

wegener granulomatosis
microscopic polyangitits
churg strauss syndrome

67
Q

direct AB mediated vasculitis

A

goodpastures

kawasaki disease

68
Q

cell meidated vasculitis

A

oran allograft rejection
inflammatory bowel disease
paraneoplastic vasculits

69
Q

unknown vasculitis

A

giant cell

takayasu arteritis

70
Q

non-specific symptoms of vasculitis

A
fever
weight loss
myalgia
arthralgia
malaise
fatigue
thrombosis
ischemia
nerve pain (depends of cause)
71
Q

large vessel vasculits

A
giant cell (temporal) arteritis
takayasu arteritis
72
Q

medium vessel vasculitis

A

polyarteritis nodosa

kawasaki disease

73
Q

small vessel vasculitis

A

wegners
churg-strauss syndrome
microscopic polyangitis

74
Q

MPO-ANCA

A

aka p-ANca

antineutrophil cytoplasmic Abs directed against myeloperoxidase

75
Q

PR3-ANCA

A

aka c-ANCA
antineutrophil cytoplasmic Abs
against proteinase 3

76
Q

giant cell arteritis

A

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

77
Q

takayasu arteritis

A

granulomatous inflammation of aorta dn brr
less then 50, females < 40
marked weakening of pulses -> aka pulseless disease) and reduced BP of UL

78
Q

aa involved w/takayasu arteritis

A
ocular
pulmonary
coronary
renal 
aortic valve insufficiency
79
Q

polyarteritis nodosa

A
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
80
Q

kawasaki disease

A

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

81
Q

kawasaki disease is associated with

A

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

82
Q

microscopic polyangitis

A

aka leukocytoclastic vascultis
necrotizing vasculitis w/few or no immune deposits (pauci immune)
p-ANCA

83
Q

presentation of microscopic polyangitis

A

palpable purpura of skin
necrotizing glomerulonephritis
hemoptysis, arthralgia, abdominal pain or bleeding, hematuria, proteinuria, hemorrhage, and m pain or weakness

84
Q

churg strauss syndrome

A
aka allergic granulomatosis and angiitis
Eos!!!!
necrotizing vasculitis
involves respiratory tract
strong association w/allergic rhinitis, bronchial asthma, lung infiltrates
blood eosinophilia
p-ANCA
85
Q

presentation of churg strauss syndrome

A

Eos!!
palpable purpura
coronary arteritis and eosinophilic myocarditis
severe renal disease infrequent

86
Q

bahcet disease

A

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

87
Q

Wegeners

A

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

88
Q

Clinical presentation of wegeners

A
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
89
Q

Thromboangiitis olterans

A

aka beurgers disese
semental thrombosin, acute and chronic inflammation of medium sized an small aa (tibial, radial) extending into vv and nn of extremities

90
Q

buerger disease presentation

A
cigarette smokers
<35
chronic ulceration, toes, feet, fingers, and gangrene
secondary raynauds
exercise induced instep claudication
severe pain, even at rest
stop smoking
91
Q

infectious vasculitis

A

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

92
Q

frostnip

A

vasoconstriction fingers, nose, toes-> discoloration

93
Q

chilblain

A

aka perniones/perniosis
due to non-freezing temps and damp conditions
chronic, recurrent vasculitis w/red raised lesions

94
Q

immersion foot

A

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

95
Q

frostbite

A

sudden sharp drops in temp
vasoconstriction and increased viscosity of blood
hyperemia and edema
large clear blisters, vesicles filled w/hemorrhagic fluid
dry gangrene

96
Q

primary raynauds

A

cold or emotion induced vasoconstirction
late in course inimal thickening can occur
median age 14
distal- proximal: blue-white-red

97
Q

secondary raynauds

A

SLE, systemic sclerosis, atherosclerosis, buergers

98
Q

phelbosclerosis

A

elastic tissue degredation and spotty calcifications w/in media
can occur w/varicose vv

99
Q

superior venal caval syndrome

A

usually neoplasm compresses or invades w/cyanosis and dilation of vv of head, neck, and arms
if pulmonary vessels also compressed causes respiratory distress

100
Q

inferior venal caval syndrome

A

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

101
Q

lymphangitis

A

infections involving lymphatics w/red streaks
group A beta hemolytic strep
in severe cases produces cellulits or focal abscesses

102
Q

acute lymphadenitis

A

red streaks w/painful enlarged regional lymph nodes

103
Q

obstructive (secondary) lymphedema

A

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

104
Q

primary lymphedema

A
  • 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
105
Q

chylous ascites, chylothorax, chylopericardium

A

caused by rupture of obstructed lymphatics into peritoneum, pleural cavity, or pericardium

106
Q

benign neoplasms

A

hemangiomas (capillary, cavernous, pyogenic granuloma)
lymphangioma (simple/capillary, cavernous)
glomus tumors

107
Q

non-neoplasm benign issues

A
vascular ectasias (nevus flammeus, spider telangiectasia, hereditary hemorrhagic telangiectasis)
reactive vascular proliferations (bacillary angiomatosis)
108
Q

intermediate grade- malignant neoplasms

A

kaposi sarcoma

hemagioednothelioma

109
Q

malignant neoplasms

A

agniosarcoma

hemagiopericytoma

110
Q

hemangiomas

A

angiomatosis- large segment of body
malignant transformations are rare
7% of all benign tumors in infancy and childhood
common in skin and liver

111
Q

capillary hemangiomas

A
  • 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
112
Q

juvenile hemangioma

A

strawberry type
capillary hemagiomas of skin in newborns
grows rapidly in first few months and regresses by age 7 in 75-90%

113
Q

cavernous hemangioma

A

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

114
Q

hemagioblastomas

A

+/- cavernous hemagiomas in von hippel lindau disease
angiomatous lesions cerebellum, brainstem, and eye
cystic neoplasms in pancreas and liver
other visceral neoplasms

115
Q

pyogenic granuloma

A
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
116
Q

granuloma gravidarum

A

pyogenic granuloma that occurs in gingiva of 1% of pregnant women
regresses postpartum

117
Q

lymphangioma circumscriptum

A

aka capillary/simple lymphangioma
small lymphatic channels
tend to occur subQ in head and neck and axilla
absence of luminal lymphangioma

118
Q

cavernous lymphangioma

A

aka cystic hygroma
in children in neck or axilla, and rarely retroperitoneal
massively dialted cystic lymphatic spaces
occur in turners syndrome
genetic testing asap

119
Q

glomus tumors

A
aka glomangioma
benign 
PAINFUL
modified cells of glomus body
(anteriovenous anasomosis)
mostly in distal digits
small, elevated, rounded, red-blue, firm nodules
120
Q

telangiectasia

A

congenital anomaly or acquired permanent exaggeration of preformed vessels

121
Q

nevus flammeus

A

birthmark
most common form of sctasia
port wine stains- usually don’t fade)
sturge-weber syndrome

122
Q

sturge-weber syndrome

A

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

123
Q

spider telangiectasia

A

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

124
Q

hereditaty hemorrhagic telangiectasia

A

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

125
Q

bacillary angiomatosis

A
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
126
Q

bacillary peliosis

A

closely related to bacillary angiomatosis vascular lesion

liver and spleen

127
Q

kaposi sarcoma

A
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
128
Q

kaposi stages

A

patch stage
plaque stage
nodular stage

129
Q

patch stage

A

pink to purple solitary or multiple macules

dilated angulated blood vessels and chronic inflammatory cells

130
Q

plaqe stage

A

larger, violaceous dermal jagged vascular channels, lined by plump spindle cells w/occasional mitoses w/RBC extravasation

131
Q

nodular stage

A

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

132
Q

types of kaposi sarcoma

A

chronic/classic/european
lymphadenopathic/african/endemic
transplant/immunosupressed associated
AIDS- associated

133
Q

chronic KS presentation

A

-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

134
Q

chronic KS treatment

A

resection of primary lesions and recurrences
radiation
chemotherapy for extensive or disseminated disease

135
Q

lymphadenopathic KS

A

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

136
Q

lymphadenopahtic KS treatment

A

chemo or radiation

137
Q

transplant associated KS

A
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
138
Q

transplant KS treatment

A

withdrawl or reduction of immunosuppressives

chemo, radiation

139
Q

AIDs associated KS

A

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

140
Q

AIDs associated KS treatment

A

antiretroviral therapy

angiogenesis inhibitors may be helpful

141
Q

hemangioendothelioma

A

intermediate btwn hemangiomas and angiosarcomas

142
Q

epithelioid hemangioendothelioma

A

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

143
Q

angiosarcoma

A

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

144
Q

cutaneous angiosarcoma

A

small often asymptomatic, red nodules

eventually become large, fleshy, pale, gray-white, with central softening, necrosis, and hemorrhage