Pulmonary Diseases of Vascular Origin

Question 1

What is the definition of an embolism?

Answer 1

A thrombus that has moved from its original location

can also refer to a foreign body

Question 2

What is a clot?

Answer 2

blood that escapes in a space/potential space (ie.subdural region)

Question 3

What pathology do you notice about the provided image?

Answer 3

thrombo emboli packed into the vessels

Question 4

What is the “Lines of Zahn”?

Answer 4

layering of the thromboemboli within the vessel rather than homogeous

lighter: fibrin & platelets
darker: erythrocytes

Question 5

What is usually the underlying reason a pulmonary embolism occurs?

Answer 5

usually predisposing condition that results in hypercoaguable state

Question 6

What are primary conditions that lead to hypercoaguable state?

Answer 6

• factor V Leiden mutation
• prothrombin mutation
• antiphospholipid syndrome

Question 7

What are secondary conditions that lead to hypercoaguable state?

Answer 7

• obesity
• recent surgery
• cancer
• oral contraceptive use
• pregnancy
• immobilization
• burns
• trauma
• fractures

Question 8

What are the gross findings associated with pulmonary embolism leading to infarction?

Answer 8

• wedge-shaped & extend to periphery o lung
• initially red-blue
  
  • the paler & red-brown after RBC lyse and hemosiderin is produced
  • eventually a scar

Question 9

What are 5 gross features that are indicative of a pulmonary embolism upon autopsy?

Answer 9

1. distends vessel
2. adherent to vessel
3. rough surface
4. Lines of Zahn
5. may or may not take shape of vessel

Question 10

What is shown in the provided image?

Answer 10

saddle clot pulmonary embolism

Question 11

What pathology is shown in the provided image?

Answer 11

pulmonary infarct

Question 12

What embolism feature is indicated by the arrows in the provided image?

Answer 12

Lines of Zahn

Question 13

What are the small white areas shown in the provided thromboembolus & how are they formed?

Answer 13

fibroblasts come into the thrombus from the vessel wall, they will cluster together and make a lining (neovascularization), hopefully restoring blood flow

Question 14

What is abnormal about the provided pulmonary tissue?

Answer 14

Infarct

very eosinophilic, no nuclei, only remnant of alveolar tissue

Question 15

What are the 5 uncommon types of Pulmonary Embolisms?

Answer 15

1. Fat and bone marrow
2. Air embolism
3. Septic embolism
4. Tumor embolism
5. Amniotic fluid embolism

Question 16

What are the main causes of bat & bone marrow embolisms?

Answer 16

trauma

after chest compression

fat embolisms after long bone or pelvic fractures

Question 17

What are the main causes of air pulmonary embolisms?

Answer 17

trauma

surgery

IV catheters

Question 18

What are the causes of septic pulmonary embolism?

Answer 18

tricuspid valve vegetation

neutrophilic inflammatoyr reaction

Question 19

What type of embolism is shown in the provided image?

Answer 19

fat/bone marrow embolism

Question 20

What substance is the clear circle in the provided H&E stain?

This is suggestive of what pathology?

Answer 20

fat

fat embolism if it is in a vessel

Question 21

What is the most common genetic muation seen in idiopathic pulmonary arterial hypertension?

Answer 21

bone morphogenic proetin receptor type 2

BMPR2

autosomal dominance with incomplete penetrance

Question 22

What are the common gross findings associated with pulmonary hypertension?

Answer 22

pulmonary artery atherosclerosis

right ventricular hypertrophy

Question 23

What microscopic findings would you expect to see in someone with pulmonary hypertension?

Answer 23

• medial hypertrophy of arterioles & small arteries
• intimal fibrosis (pinpoint lumen)
• atheromatous deposits in pulmonary artery & major branches
• plexiform lesions

Question 24

If you find many organized & recanalized thrombi, what was probalby the cause of the pulmonary hypertension?

What if it is present with emphysema & chronic bronchitis?

Answer 24

due to chronic thromboemboli

duet to COPD

Question 25

What demographic is most commonly affected by idiopathic pulmonary hypertension? Symptom progression? Treatment?

Answer 25

* Demographic * 20-40 yr * women * Symptoms * initially * dyspnea, fatigue, angina * later * severe respiratory distress * right ventricular hypertrophy * cor pulmonale * Treatment * therapy for driggers * vasodilators * lung transplant

Question 26

What pathology is demonstrated by the provided histological slide?

Answer 26

Diffuse pulmonary hemorrhage syndrome lots of blue (positive for iron) macrophages that have been breaking down erythrocytes - indicates the process has happened over time or repetitively

Question 27

What are the major pathological components of Goodpasture syndrome? What is the most commonly affected demographic? Treatment?

Answer 27

* Lung & kidney injury b/c autoantibodies against **type IV collagen a3 chain** * inflammatory destruction of basement membranes in glomeruli & alveoli * hemoptysis * rapidly progressive glomerulonephritis * necrotizing hemorrhageic interestitial pneumonitis * Demographics * teens or 20s * males * smokers * Treatment * plasmapheresis - removes antibodies * immunosupression

Question 28

What are the gross findings from the lung of a person with Goodpasture synrome?

Answer 28

heavy lungs with red-brown consolidation

Question 29

What are the microscopic findings from the lung of a person with Goodpasture synrome?

Answer 29

* necrosis alveolar walls * alveolar hemorrhage * hemosiderin-laden macrphages in alveoli (filled w/ iron) * septal fibrosis, type II pneumocyte hypertophy * immunofluorescent linear Ig deposits along septal wall basememtn membranes

Question 30

What pathology is shown by the provided histological slide? How do you know?

Answer 30

**Goodpasture's syndrome** thickened alveolar walls, prominent type Ii pneumocytes, many RBC & macrophages in alveoli higher magnification provided

Question 31

What feature of Goodpasture's syndrome is beign depicted by the provided image?

Answer 31

linear immunoglobulin deposits along alveolar walls

Question 32

What is the pathogenesis of idiopathic pulmonary hemosiderosis? Clinical presentation? Most commonly associated demographic? Treatment?

Answer 32

* Pathogenesis * intermittenet diffue alveolar hemorrhage * pulmonary findings similar to Goodpasture * Clinical presentation * cough * hemoptysis * anemia * Demographic * young children * Treatment * immunosuppressive therapy

Question 33

What is the pathogenesis of Polyangiitis with ganulomatosis? Associated mutation? Clnical presentation?

Answer 33

* Pathogenesis * autoimmune disease of upper respiratory tract and/ or lungs * T-cell mediated hypersensitivity to inhaled agents * **PR3-ANCA (C-ANCA)** * Necrotizing vasculitis * granulomas of repiratory tract * granulomas of small to medium vessels * focal nectorizing glomerulonephritis * Clinical presentation * hemoptysis * inflammatory sinusitis with mucosal granulomas * ulcers of respiratory tract with associated granulomas * alveolar hemorrhage

Question 34

What pathology is shown in the provided images?

Answer 34

Polyangiitis with ganulomatosis * Left: * multinucleated histiocytes * poorly formed granuloma on bottom left * lots of inflammation in vessel wall (vasculitis) * Right: * large nodular centrally cavitating lesions

Question 35

The geographic pattern shown in the provided slides is suggestive of what diagnosis?

Answer 35

Polyangiitis with ganulomatosis necrosis (dark blue) & granulomas (circular) lots of multinucleated cells