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System Pathology I -- Roecker > Exam 3: Vascular Pathology Pt.3 > Flashcards

Exam 3: Vascular Pathology Pt.3 Flashcards

1
Q

What is a form of pediatric vasculitis that we think has an autoimmune origin and is believed to be due to a viral trigger and manifests with an acute fever and large aneurysms in the coronary arteries?

A

Kawasaki Disease

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2
Q

In what disease does a young child have a “strawberry tongue”, swollen extremities, cervical lymphadenopathy and in 20% of cases it places the child at risk for a lethal myocardial infarction?

A

Kawasaki Disease

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3
Q

How does Kawasaki Disease manifest?

A
  • widespread inflammation
  • oral erythema (“strawberry tongue”)
  • swollen extremities
  • desquamtation or blistering of erythematous skin
  • cervical lymphadenopathy
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4
Q

Does Kawasaki Disease have a genetic influence? If so, who or how?

A

yes, Asian descent are more likely to develop it

- siblings of affected ind are 10-20x more likely to develop it

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5
Q

What age group, sex, and how common is Kawasaki Disease?

A
  • exclusively young children, most under 4 yrs
  • males
  • 1 in 25,000 under age 5
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6
Q

T/F. Children that have Kawasaki disease can be given Ibuprofen or acetaminophen to help with the symptoms.

A

False– it has no response to those drugs

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7
Q

What is am extremely rare condition that occurs in children, typically 4-12 yrs, that are given aspirin while recovering from a viral infection, and is a medical emergency?

A

Reye Syndrome

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8
Q

What are the most common viral infections associated with Reye Syndrome?

A
  • influenzae

- varicella-zoster virus (chickenpox)

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9
Q

What is effected in children that develop Reye Syndrome?

A 
Liver --> steatosis and hepatic dysfunction 
Brain --> encephalopathy
- seizures
- confusion
- lethargy
- loss of consciousness
- coma/death
  • inflammatory reaction
  • vomiting
  • diarrhea
  • rashes
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10
Q

What is a T cell-mediated, type IV hypersensitivity, that causes destructive “necrotizing granulomas” w/in kidneys, lungs, trachea, and sinuses, and causes hemoptysis and hematuria?

A

Wegener Granulomatosis

hemoptysis = coughing up blood

hematuria = blood w/in urine

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11
Q

What four main things does Wegener Granulomatosis cause?

A
  1. chronic granulomatous inflammation
  2. widespread necrotizing vasculitis
  3. kidney damage–> glomerulonephritis
  4. pneumonitis
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12
Q

What individuals is Wegener Granulomatosis most likely to develop in and what may it cause within 1 year?

A

middle-aged males and cause lethal kidney damage w/in 1 year of most patients (80%)

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13
Q

What is the most common manifestation of Wegener granulomatosis and what is it associated with?

A

pneumonitis: ass. with:
- cough
- chest pain
- dyspnea
- hemoptysis

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14
Q

What other disease does Wegener Granulomatosis have a similar manifestation as? What makes them different?

A

Polyarteritis nodosa

– but Wegener Granulomatosis involves lungs and upper respiratory tract or sinuses

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15
Q

What vasculitis disease is directly related to tobacco smoking and is believed to develop in genetically predisposed inds?

A

Buerger Disease

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16
Q

How does Buerger Disease manifest in its earlier stages? What about the advanced stages?

A
  • cold and cyanotic extremitis
  • vascular claudication
  • Raynaud phenomenon
  • soft tissue ulcerations
  • gangrenous necrosis
  • leads to amputation
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17
Q

Describe that onset of Buerger Disease. What arteries are effects?

A

acute onset causing severe transmural vasculitis in small-to-medium arteries of extremities:
- tibial arteries and radial arteries

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18
Q

What may help Buerger Disease to improve? How long does it take for severe ischemia and permanent vascular occlusion to occur if one continues to smoke?

A

STOP smoking

if continue–> that may occur in 5-8 years

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19
Q

What age group is characteristically affected by Buerger disease? What sex? Any ethnicity have greater risk?

A

males 3x more likely
b/w ages 20-40 and heavy cigarette smokers

Asians (japan, korea, natives of india) and Ashkenazi jews

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20
Q

What condition develops following inappropriate and exaggerated vasoconstriction causing pallor or cyanosis of fingers?

A

Raynaud Phenomenon

may be Primary or Secondary

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21
Q

What may occur in the most severe or chronic cases of Raynaud phenomenon?

A

atrophy of skin, subcutaneous tissues, and underlying musculature

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22
Q

How does one get Primary Raynaud Phenomenon? What triggers it and who is most common affected?

A

idiopathic and triggered by cold exposure and emotional stress

young adult females

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23
Q

What conditions may cause Secondary Raynaud Phenomenon?

A
  • Buerger Disease
  • lupus (SLE)
  • CREST syndrome
  • severe atherosclerosis
  • after smoking
  • following trauma
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24
Q

What is an acute coronary syndrome that develops following excessive and prolonged coronary artery vasospam and develops when the individual experiences extreme emotional distress?

A

Takotsubo Cardiomyopathy (cardiac Raynaud’s or Stress-induced cardiomyopathy or Broken heart syndrome”

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25
Q

Takotsubo Cardiomyopathy has been reported to develop following what situations?

A
  • learning of death of a loved one
  • motor vehicle accidents
  • learning of significant diagnosis/illness
  • being surprised
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26
Q

What group of individuals is Takotsubo cardiomyopathy more common in? (age, sex, race)

A
  • older adult postmenopausal females

- Asian or Caucasian inds

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27
Q

What causes the heart injury associated with Takotsubo cardiomyopathy?

A

emotional event–> causes release of catecholamines into blood –> causes severe cardiac ischemia and prolonged coronary artery vasospam

catecholamines increase HR and strength of contraction and combined with the ischemia = injury

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28
Q

What do individuals who experience Takotsubo cardiomyopathy suffer from?

A

prolonged arrhythmia–> typically ventricular fibrillation, and experience sudden cardiac death

may have M.I. if ischemia persists for duration of 20-30 mins

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29
Q

What is the term for “inflammation of a vein”, but is also used to describe inflammatory conditions that cause blood to clot w/in a vein? What is the MC form?

A

Thrombophlebitis

Deep Vein Thrombosis (90%)

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30
Q

What term describes when blood clots within a vein, WITHOUT presence of inflammation?

A

Phlebothrombosis

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31
Q

What are dilated and tortuous superficial veins that MC are located in the leg and thigh?

A

Varicose veins

smaller ones –> telangiectasia or “spider veins”

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32
Q

What causes varicose veins?

A

following chronic intravascular pressure in veins of lower extremities causing:

  • valvular incompetence
  • stasis and congestion of venous blood
  • pitting edema
  • pain
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33
Q

T/F. Emboli frequently develop at the site of varicose veins.

A

False (even though they do frequently develop in deep veins of lower extremity)

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34
Q

What is the average age of Dx for varicose veins? What fraction of females and males get them?

A

age 55

1/3 of all women
1/5 all males

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35
Q

What are risk factors for developing varicose veins?

A
  • obesity
  • having been pregnant
  • wearing high-heeled footwear
  • having a family Hx
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36
Q

What may occur with severe cases of venous congestion due to more sever varicose veins?

A
  • poor wound healing
  • ulcerations (“varicose ulcers”)
  • infections
  • stasis dermatitis
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37
Q

What is a chronic inflammatory skin condition that develops on the lower extremities following periods of prolonged lower extremity venous insufficiency?

A

Stasis dermatitis (venous eczema)

38
Q

What is the chronic inflammatory skin conditions, Stasis dermatitis, believed to develop from?

A
  • harmful effects of increased venous pressure
  • reduced tissue perfusion/hypoxia
  • increase in matrix metalloproteinase (MMP)
39
Q

What individuals is Stasis Dermatitis most likely to develop in?

A

if have varicose veins and affects older adults

40
Q

What is the appearance of the skin of someone with Stasis Dermatitis?

A
  • more pigmented or erythematous appearance

- thickening of skin or flaking

41
Q

What are complications of Stasis Dermatitis?

A
  • pain
  • cosmetic concerns
  • nonhealing cutaneous ulcers–> may cause bacterial skin infs (i.e. cellulitis)
42
Q

What is Superior Vena Cava Syndrome?

A

develops when blood flow through SVC is obstructed

43
Q

What are causes for Superior Vena Cava Syndrome? Which two are the MC reasons?

A
  • lung cancer (MC)
  • implanted hardware (pacemaker)
  • mediastinal lymphoma (MC)
  • thrmobosis w/in SVC
44
Q

What are the clinical features associated with Superior Vena Cava Syndrome?

A
  • venous distention of head, neck, and upper extremities
  • Congested Upper Extremity Veins = UE cyanosis/edema
  • HAs
  • dizziness
  • facial swelling
45
Q

Which is MC, Superior or Inferior Vena Cava Syndrome?

A

Inferior Vena Cava syndrome is MC

46
Q

What develops when blood flow through the inferior vena cava is obstructed?

A

Inferior Vena Cava Syndrome

47
Q

What are causes of Inferior Vena Cava Syndrome?

A
  • Hepatocellular carcinoma (liver CA)
  • Thrombus of lower extremity veins (DVT)
  • renal cell carcinoma (kidney CA)
  • pregnancy
48
Q

What are the clinical features of Inferior Vena Cava Syndrome?

A
  • pitting edema in lower extremities
  • lower extremity cyanosis
  • kidney dysfunction –> proteinuria
49
Q

What is Lymphedema?

A

an abnormal collection of fluid w/in interstitium and lymphatic channels, resulting from an obstruction of lymphatic drainage

congenital or acquired

can be Primary or Secondary

50
Q

What occurs distal to the obstruction in Lymphedema?

A
  • lymphatic channels have elevated hydrostatic pressure
  • interstital fluid accumulates
  • tissues become engorged
  • peau d’orange or brawny induration
51
Q

What is a thickening and hardening of the skin due to fibrosis at the site of skin that has been chronically inflamed? (is ass. with Lymphedma)

A

Brawny induration

52
Q

What is a rare inherited congenital condition that develops following the agenesis or hypoplasia of lymphatics?

A

Primary Lymphedema (aka Milroy disease)

53
Q

What causes Milroy Disease?

A

(primary lymphedema)

  • autosomal dominant condition due to mutation in FLT4 gene
  • interfere with formation of endothelial lining of lymphatic system and absent or underdeveloped lymphatics
54
Q

Which is MC, primary or secondary lymphedema?

A

secondary lymphedema

55
Q

How does Secondary Lymphedema develop? What are common stimuli for it?

A

following inflammation/fibrotic, infectious, or neoplastic diseases

  • tumors
  • surgery-related scarring
  • radiation-related scarring
  • inflammation-related blood clots or scarring
  • filariasis-related scarring
56
Q

What is an acute inflammation of the lymphatic channel due to infectious process?

A

Lymphangitis (“bacterial lymphangitis”)

57
Q

What do most cases of Lymphangitis develop from?

A

bacterial infection due to minor skin trauma

Streptococcal and Staphylococcal organisms are common causes

58
Q

What are characteristics of Lymphangitis?

A
  • acute inflammation
  • painful subcutaneous red streaks in area of lymph channel
  • fever, chills, malaise, HA, anorexia, muscle aches
  • possible bacteremia and SEPSIS
59
Q

What do vascular tumors develop from?

A

vascular endothelial cells (MC) and surrounding vascular CT

most are benign

60
Q

What are benign tumors of capillary endothelial cells and are well-differentiated with little-to-no risk of malignant transformation?

A

Hemangioma

61
Q

Where are hemangiomas mostly discovered? But where do 30% of all hemangiomas?

A

on the skin of face, neck, head of young children

but 30% are located with the liver

62
Q

What are the common “strawberry hemangiomas” discovered early in life and are present in 1 in 200 newborns called? How do they manifest?

A

Juvenile hemangiomas

grow rapidly for few months–> by age 1-3 start to fade –> then ~90% self-resolve w/in 1st decade of life

63
Q

Whar are hemangiomas that develop on the gingiva, oral mucosa, or skin of pregnant women? What are they thought to be due to?

A

Pyogenic granulomas

hormonal fluctuations of pregnancy–> therefore sometimes called “pregnancy tumors”

64
Q

What are hemangiomas that develop w/in bone and are MC discovered w/in bones of spine or skull?

A

Interosseous hemangiomas

65
Q

Where are Interosseous hemangiomas most likely to develop?

A

in spine or skull

-when in spine–> commonly involve single VB–> called “vertebral hemangioma”

66
Q

What percent of the population do Vertebral hemangiomas affect? What sex is more commonly affect?

A

10% of adult population

males 2x

no clinical significance

67
Q

What is a lymphatic endothelial tumor that develops following a Kaposi sarcoma herpesvirus infection?

A

Kaposi Saracoma

68
Q

What are an AIDS-defining illness and is much more likely to develop among individuals in the crisis phase of AIDS?

A

Kaposi Sarcoma

69
Q

What is characteristically required for one to develop Kaposi Sarcoma?

A

The Kaposi Sarcoma Herpesvirus typically becomes latent and the combination of this virus and immunosuppression s required

(there AIDS inds)

70
Q

How do the areas of Kaposi Sarcoma appear? Where are they MC?

A
  • raised pink, red, or purple patch or patches that form from the vascular tumor (Maculas–> “purpura”)
  • often raised and asymptomatic lesions
  • endothelial cells are irregular and high amounts of inflammatory cells

MC on Lower Extremities

71
Q

What is the MC HIV-related malignancy?

A

Kaposi Sarcoma

very aggressive

72
Q

What are malignant endothelial tumors of vasculature that contain fairly well-differentiated endothelial cells or highly anaplastic endothelial cells?

A

Angiosarcoma

high anaplastic–> is aggressive and it is assumed to be this until proven otherwise

73
Q

How are Angiosaracomas able to spread? Where do they most likely spread to?

A

spread via hematogenous or lymphatic systems–> to lungs or liver

74
Q

What is the prognosis for anaplastic angiosarcomas?

A

poor, about 30% of all inds survive another 5 years after Dx

75
Q

Where are angiosarcomas most commonly discovered?

A
  • skin
  • w/in breast
  • w/in various soft tissues
  • w/in liver

(but could be anywhere with vascularity)

76
Q

How do angiosarcomas that develop on the skin manifest?

A
  • small well-documented red nodules
  • asymptomatic, but may have pain or itching
  • as larger, they manifest with tissue necrosis or hemorrhage
77
Q

What are risk factors for developing angiosarcomas?

A
  • soft tissue trauma (surgery or foreign body)

- exposure to env. poisons (arsenic or PVC)

78
Q

What are Hepatic angiosarcomas characteristically associated with?

A

shaving Hx of working in PVC manufacturing industry–> long latency period and will develop several years after exposure

79
Q

What is the placement of an intravascular stent known as?

A

endovascular stenting

80
Q

What is a stent?

A

a tubular support structure that is inserted into blood vessel to maintain the patency of the vascular lumen at site of arterial stenosis

81
Q

In what patients is Endovascular stenting a very common procedure for? What else can it be used for?

A
  • common in patients with–> coronary artery disease
    ALSO for:
  • hold down intimal flaps in someone w/ arterial dissection
  • limit vasospasm in someone with Prinzmental angina
82
Q

What is the term for the condition of being open, expanded, or unobstructed?

A

patency

83
Q

How is endovascular stenting typically performed?

A

following expansion of a balloon angioplasty to open the site of vascular stenosis

84
Q

What is the term for replacing or bypassing a diseased vessel?

A

Vascular Grafting

like to bypass an artery involved in CAD

85
Q

What are two things that vascular grafts can be composed of?

A
  1. Synthetic material

2. Autologous grafts (autologous = cells or tissues obtained from the same ind)

86
Q

What vessels are commonly used for Autologous grafts?

A

great saphenous vein or left internal mammary artery

87
Q

What is the name of the procedure when vascular grafts are used to manage CAD?

A

coronary artery bypass graft (CABG, pronounced like “cabbage”)

88
Q

What is the limited long-term utility of using the great saphenous vein for a vascular graft? what about for the internal mammary artery?

A

great saphenous vein–> 50% chance of lasting 10 years

internal mammary artery–> 90% chance of lasting 10 years

89
Q

What are compression stockings used for?

A
  • encourage venous return

- external pressure to minimize lymphedema

90
Q

What else should one do that is using compression stockings to help the swelling of legs?

A
  • leg elevation
  • recommendations to stay active
  • weight loss
  • manual lymphatic drainage massage
  • regular skin evaluations

System Pathology I -- Roecker (14 decks)

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