Blood Vessels

Question 1

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

Answer 1

Developmental/berry aneurysms

Question 2

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

Answer 2

Arteriovenous fistulas

Question 3

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 3

Fibromuscular dysplasia

Question 4

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 4

Hyaline arteriolosclerosis

Question 5

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 5

Hyperplastic artieriolosclerosis

Question 6

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

Answer 6

Atherosclerosis

Question 7

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 7

Fatty streaks

Question 8

Three principal components of an atheromatous plaque.

Answer 8

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

Question 9

Most common site of atherosclerosis.

Answer 9

Abdominal aorta

Question 10

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

Answer 10

Stable plaque

Question 11

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

Answer 11

Vulnerable plaque

Question 12

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

Answer 12

Rupture, ulceration or erosion

Question 13

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

Answer 13

Hemorrhage

Question 14

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

Answer 14

Atheroembolism

Question 15

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

Answer 15

Aneurysm formation

Question 16

Critical stenosis in coronary arteries

Answer 16

70%

Question 17

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

Answer 17

Aneurysm

Question 18

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

Answer 18

True aneurysm

Question 19

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

Answer 19

False aneurysm

Question 20

Two most important causes of aortic aneurysms.

Answer 20

Atherosclerosis (AAA) and hypertension (Ascending aortic aneurysm)

Question 21

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 21

Abdominal aortic aneurysm (AAA)

Question 22

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 22

Syphilitic aortitis

Question 23

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

Answer 23

Arterial dissection

Question 24

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 24

Cystic medial degeneration

Question 25

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:

Answer 25

Giant cell arteritis (Large arteries)

Question 26

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:

Answer 26

Takayasu arteritis (Large arteries)

Question 27

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:

Answer 27

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 28

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:

Answer 28

Kawasaki disease (Medium-sized)

Question 29

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 29

Microscopic polyangiitis (Small arteries)

Question 30

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).

Answer 30

Churg-Strauss syndrome (Small arteries)

Question 31

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 31

Wegener granulomatosis

Question 32

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 32

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

Question 33

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 33

Raynaud Phenomenon

Question 34

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 34

Varicose veins

Question 35

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

Answer 35

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

Question 36

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 36

Superior vena cava syndrome

Question 37

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 37

Inferior vena cava syndrome

Question 38

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

Answer 38

GABHS

Question 39

Most common form of vascular ectasia.

Answer 39

Nevus flammeus (birthmark)

Question 40

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

Answer 40

Spider telangiectasia

Question 41

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 41

A (Diagnosis is Sturge-Weber syndrome or encephalotrigeminal angiomatosis)

Question 42

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 42

Hereditary hemorrhagic telengiectasia/Osler-Weber-Rendu disease

Question 43

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 43

Capillary hemangiomas

Question 44

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 44

Cavernous hemangioma

Question 45

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 45

Kaposi sarcoma

Question 46

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 46

Angiosarcoma