10 - Blood vessels

Question 1

Enumerate (2): most common causes of death in hypertension:

Answer 1

• hypertensive heart disease
• ischemic heart disease

Question 2

Most common cause of aortic dissection:

Answer 2

intimal tear

Question 3

Most dangerous consequence of acute plaque change (atherosclerotic disease)

Answer 3

abrupt cessation of blood flow

Question 4

Most common cause of death in aortic dissection:

Answer 4

rupture

Question 5

Most common form of vasculitis among older individuals:

Answer 5

giant cell arteritis

Question 6

Triad of Behcet syndrome (3)

Answer 6

• recurrent oral aphthous ulcers
• genital ulcers
• uveitis

Question 7

Most common cause of of lymphangitis:

Answer 7

group A beta-hemolytic strep

Question 8

These are small spherical dilatations, typically in the circle of Willis.

Answer 8

Developmental/berry aneurysms

Question 9

These are abnormal, typically small, direct connections between arteries and veins that bypass the intervening capillaries.

Answer 9

Arteriovenous fistulas

Question 10

Focal, irregular thickening of the walls of medium and large muscular arteries; segments of the vessel wall are focally thickened by combination of irregular medial and intimal hyperplasia and fibrosis, causing luminal stenosis.

Answer 10

Fibromuscular dysplasia

Question 11

Consists of a homogenous pink hyaline thickening of the walls of arterioles with loss of underlying structural detail and with narrowing of the lumen; major morphologic characteristic in benign nephrosclerosis.

Answer 11

Hyaline arteriolosclerosis

Question 12

Characteristic of malignant hypertension, associated with onion-skin concentric, laminated, thickening of the walls of arterioles with luminal narrowing; these laminations consist of smooth muscle cells and thickened duplicated basement membrane; associated with necrotizing arteriolitis.

Answer 12

Hyperplastic artieriolosclerosis

Question 13

Characterized by intimal lesions called atheromas that protrude into vascular lumina.

Answer 13

Atherosclerosis

Question 14

Composed of lipid-filled macrophages and smooth muscle cells (foam cells) but are not significantly raised and thus do not cause any disturbance in blood flow; can appear as early as 1 year, and present in virtually all children older than 10 years old.

Answer 14

Fatty streaks

Question 15

Three principal components of an atheromatous plaque.

Answer 15

1. Cells (SM cells, macrophages, T cells)
2. Extracellular matrix (collagen, elastic fibers, proteoglycans); and
3. Intracellular and extracellular lipid

Question 16

Most common site of atherosclerosis.

Answer 16

Abdominal aorta

Question 17

Plaque that has a thick fibrous cap, minimal lipid core, and minimal inflammation.

Answer 17

Stable plaque

Question 18

Plaque that has a thin fibrous cap, large lipid core, and greater inflammation.

Answer 18

Vulnerable plaque

Question 19

The luminal surface exposes the bloodstream to highly thrombogenic substances and induces thrombus formation.

Answer 19

Rupture, ulceration or erosion

Question 20

Rupture of the overlying fibrous cap or the thin-walled vessels in the areas of neovascularization, results in:

Answer 20

Hemorrhage

Question 21

Discharge of debris into the bloodstream, producing microemboli composed of plaque contents.

Answer 21

Atheroembolism

Question 22

Increased pressure or ischemic atrophy of the underlying media, with loss of elastic tissue, leading to weakness of the vessel wall.

Answer 22

Aneurysm formation

Question 23

Critical stenosis in coronary arteries

Answer 23

70%

Question 24

It is a localized abnormal dilation of a blood vessel or heart.

Answer 24

Aneurysm

Question 25

Aneurysm that involves all three layers of the arterial wall, or the attenuated wall of the heart.

Answer 25

True aneurysm

Question 26

A breach in the vascular wall leading to an extravascular hematoma that freely communicates with the intravascular space.

Answer 26

False aneurysm

Question 27

Two most important causes of aortic aneurysms.

Answer 27

Atherosclerosis (AAA) and hypertension (Ascending aortic aneurysm)

Question 28

This disease can more commonly affects men greater than 50 years old. Lesion usually positioned below the renal arteries and above the aortic bifurcation; can be saccular (dilation of one portion) or fusiform (circumferential dilation).

Answer 28

Abdominal aortic aneurysm (AAA)

Question 29

Small blood vessels and vasa vasorum show luminal narrowing and obliteration (Obliterative endarteritis) scarring of the vessel wall and a dense surrounding rim of lymphocytes and plasma cells that may extend into the media; characteristic of the tertiary stage of Syphilis.

Answer 29

Syphilitic aortitis

Question 30

Arises when blood enters the wall of an artery, as a hematoma dissecting between its layers; often, but not always aneurysmal in origin.

Answer 30

Arterial dissection

Question 31

Most frequent pre-existing histologically detectable lesion in aortic dissection; characterized by elastic tissue fragmentation and separation of the elastic and smooth muscle cell elements of the media by cystic spaces filled with amorphous proteoglycan-rich extracellular matrix.

Answer 31

Cystic medial degeneration

Question 32

• 38/M presents with severe headache and diplopia associated with fever and fatigue. Area along the course of the superficial temporal artery was painful to palpation. Biopsy done revealed intimal thickening of the artery, granulomatous inflammation of the internal elastic lamina with infiltrate of T cells and macrophages. The most likely diagnosis is:
• Size of affected vessels:

Answer 32

Giant cell arteritis (Large arteries)

Question 33

• Patient initially present with fatigue, weight loss and fever and then reduced blood pressure and weak pulses in the carotids and upper extremities, ocular disturbances and neurologic deficits. The most likely diagnosis is:
• Size of affected vessels:

Answer 33

Takayasu arteritis (Large arteries)

Question 34

• This disease presents with rapidly accelerating hypertension, abdominal pain, bloody stool, myalgia and peripheral neuritis; histologically, there is segmental transmural necrotizing inflammation that spares the pulmonary circulation with extensive fibrinoid necrosis and temporal heterogeneity of lesions. The most likely diagnosis is:
• Size of the affected blood vessels?

Answer 34

Polyarteritis nodosa (Small to medium-sized arteries)

Remember: PAN can be renal or visceral, but never pulmonary

Memory device: PAN in small arteries that are temporally homogenous (lesions are of same age)

Question 35

• 4/F presented with fever and maculopapular rash on her trunk and extremities. This is associated with conjunctival erythema, edema of the hands and feet, and cervical lymph node enlargement; on biopsy, there is segmental transmural necrotizing inflammation with less fibrinoid necrosis. The most likely diagnosis is:
• Size of affected vessels:

Answer 35

Kawasaki disease (Medium-sized)

Question 36

A necrotizing vasculitis that generally affects capillaries, arterioles and venules, with few or no immune deposits; necrotizing glomerulonephritis and pulmonary capillaritis are common; associated with p-ANCA (MPO-ANCA); also exhibits leukocytoclasia (fragmented PMNs in post-capillary venules); thus also the term leukocytoclastic angiitis.

Answer 36

Microscopic polyangiitis (Small arteries)

Question 37

• Eosinophil-rich and granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small vessels; associated with asthma and blood eosinophilia; associated with p-ANCA (MPO-ANCA).
• Size of affected vessels?

Answer 37

Churg-Strauss syndrome (Small arteries)

Question 38

Causes necrotizing granulomatous vasculitis with fibroblastic proliferation; Involves small vessels most notable in the upper and lower; can cause crescentic glomerulonephritis; associated with c-ANCA (PR3-ANCA).

Answer 38

Wegener granulomatosis

Question 39

Characterized by sharply segmental acute and chronic vasculitis of medium sized and small arteries, predominantly of the extremities; there is acute and chronic inflammation accompanied by luminal thrombosis, containing microabscesses composed of neutrophils surrounded by granulomatous inflammation; strong relationship with cigarette smoking.

Answer 39

Thromboangiitis obliterans (Buerger disease)

Note: Do not confuse with Berger disease (IgA nephropathy)

BuergER is a smokER

Question 40

Results from an exaggerated vasoconstriction of digital arteries and arterioles, inducing paroxysmal pallor or cyanosis of the digits of the hands and feet; can be primary or secondary.

Answer 40

Raynaud Phenomenon

Question 41

Abnormally dilated, tortuous veins produced by prolonged increase in intraluminal pressure and loss of vessel wall support; veins show wall thinning at the points of maximal dilation with smooth muscle hypertrophy and intimal fibrosis; focal thrombosis and venous valve deformities are common; most common sites are superficial veins of the upper and lower leg.

Answer 41

Varicose veins

Question 42

Three sites of varices produced in the presence of portal hypertension.

Answer 42

GEJ (Esophageal varices), Rectum (Hemorrhoids), Periumbillical veins (Caput medusae)

Question 43

Usually arises in the setting of bronchogenic carcinoma, mediastinal lymphoma, and other SOLs (aneurysms); usually presents with dilation of the veins of the head, neck, and arms with cyanosis; respiratory distress occurs with pulmonary vessel involvement.

Answer 43

Superior vena cava syndrome

Question 44

Usually arises in the setting of tumors that invade IVC (hepatocellular and renal), and venous thrombosis; usually present with Lower extremity edema, superficial abdominal vein distention, massive proteinuria with renal vein involvement.

Answer 44

Inferior vena cava syndrome

Question 45

Most common cause of lymphangitis (usually with accompanying lymphadenitis).

Answer 45

GABHS

Question 46

• Most common form of vascular ectasia.
• Another name for these lesions:

Answer 46

• Nevus flammeus (birthmark)
• a.k.a. port-wine stain

Question 47

Radial, often pulsatile arrays of dilated subcutaneous arteries about a central core that blanch with pressure; associated with hyperestrinism (pregnancy and liver cirrhosis).

Answer 47

Spider telangiectasia

Question 48

3/F with a large port wine stain on the right side of her face has occasional seizures. Further examination showed mental developmental delay. She may also have:

A. More extensive vascular malformations

B. Multiple colonic polyps

C. Color blindness

D. Alpha thalassemia

Answer 48

A

Diagnosis is Sturge-Weber syndrome or encephalotrigeminal angiomatosis

Question 49

An autosomal dominant disorder caused by mutations in genes that encode components of TGF-b signaling pathway. Presents with telengiectasia that are widely distributed over the skin and mucus membranes.

Answer 49

Hereditary hemorrhagic telengiectasia/Osler-Weber-Rendu disease

Question 50

These are bright red to blue lesions, that vary from a few millimeters to several centimeters in diameter; Unencapsulated aggregates of closely packed, thin-walled capillaries, usually blood-filled and lined by flattened endothelium; vessels are separated by scant connective tissue stroma; can also be filled with lymph (lymphangioma)

Answer 50

Capillary hemangiomas

Question 51

Appears as red-blue, soft, spongy masses 1-2 cm in diameter, which can affect large subcutaneous areas of the face, extremities, and other body regions; mass is sharply defined but not encapsulated, composed of large, cavernous, blood-filled spaces; can also be filled with lymph (lymphangioma).

Answer 51

Cavernous hemangioma

Question 52

Common in patients with AIDS, caused by HHV-8, causing skin lesions that progress from patches, to plaques, to nodules; there are usually plump spindle cells with extravasated erythrocytes and hemosiderin-laden magrophages in the lesions

Answer 52

Kaposi sarcoma

Question 53

These are malignant endothelial neoplasms, with varying histology from plump, anaplastic but recognizable endothelial cells producing vascular channels to widely undifferentiated tumors having solid, spindle cell appearance and producing nondefinite blood vessels; endothelial cell markers include CD31 and vWF.

Answer 53

Angiosarcoma