Vascular Lung Disease Flashcards
pulmonary embolism vs pulmonary thrombosis
- PE - comes from deep veins
- pulmonary thrombus - actually originates in the lung
“lines of zahn”
a gross or microscopic layering of a thromboembolism:
- layers are light pink and maroon
- lighter areas = fibrin, plateles
- darker areas = erythrocytes (RBCs)
PE pathogenesis
- typically, a condition that creates a hypercoagulable state
- primary conditions:
- mutations in factor V (Leiden) / prothrombin
- anti-phospholipid syndrome
- secondary conditions (more common)
- obesity
- pregnancy
- recent surgery
- oral contraceptives
- burns / trauma / fractures
- primary conditions:
where in the lung are PEs found
either in the
- PA
- embolus in bifurcation of main PA = saddle embolus (shown in pic)
- PA branches
PE - gross morphology
- embolus in PA/its branches
- possible infarct (prevented by good compensatory flow from bronchial arteries)
- at lung periphery
- “wedge shaped”
- blue red –> pale –> red brown –> scar
PE - microscopic morphology
- peripherally located infarct (note the visceral pleura bordering the infarct - this is the edge of a lung lobe)
- a hemorraghic area with ischemic necrosis
- made of ines of Zahn: laminations made of platelet/fibrin (pink) layers alternating with RBC-rich (red) layers)
contrast the morphologies of a PE and a postmortem clot
label the curved black arrow, blue circle and red circle
- black arrow/ red circle / blue circle circle = normal tissue
- black arrow = remaining bronchiole
- red / blue circle = remaining intact alveoli
- green circle = unhealthy tissue
- esionophilic
- anucleated
what are the non-blood types of PEs?
what circumstances predisposes formation of these PEs?
-
fat/bone marrow PE - post bone breakage (trauma)
- __chest compressions during CPR
- long bone / pelvic fractures
- air embolism - surgery/IV catheters
- septic embolism - from neutrophilic rxn following bacterial infection of tricuspid valve
- tumor emolism
- amniotic fluid embolism
in what population are septic embolisms most common?
- IV drug users –> tricuspid valve endocarditis
what is shown in this image?
bone marrow embolus
(common follows bone fracture/chest compressions)
what is shown in this image?
fat pulmonary embolism
(typically follows bone fractures / chest compressions)
what is shown in this image?
amniotic fluid embolism
pulmonary hypertension (PH)
- is defined as?
- pathogenesis?
- has what morphology (gross / microscopic)?
- can progress to?
- MAP > 25 mmHg
-
pathogenesis: multiple causes all of which inrease resistance in pulmonary capillaries
- COPD / recurrent thromboemboli / autoimmune vascular dz / heart
-
morphology:
- gross: PA atherosclerosis + RV hypertrophy
- microscopic:
- atheroscleromatous deposits in PA/its branches
- medial hypertrophy of arterioles/small arteries
- i_ntimal fibrosis_
- advanced cases —> plexiform lesion
- can become:
- respiratory distress
- RVH
- cor-pulmonale
atheroma found in large vessels of pt with pulmonary artery hypertension
marked medial hypertrophy of a pulmonary small artery/arteriole
PAH
Group 1 PAH
- cause
- demographics
- tx
- group 1 = “idiopathic”
- though most pts have BMPR2 mutation (bone morphogenic protein mutation)
- 20-40 yr old women
- tx: vasodilators
diffuse pulmonary hemorrhage syndromes
- what are they?
- what microscopic morphology do they share?
- three syndromes:
- goodpasture
- idiopathic pulmonary hemosiderosis
- polyangitis with granulomatosis (wegners)
- core microscopic presentation: presence of
-
intra-alveolar
- erythrocytes (RBCs)
-
hemosierin-laden macrophages
- - which stain with prussion iron blue stain
-
intra-alveolar
goodpasture syndrome
- definition
- pathogenesis
- demographics
- type of diffuse pulmonary hemorrhage syndrome
- is an auto-immune disorder: body makes Ab against type IV collagen a3 chain which is in the
- LUNGS –> leads to rapid progressive glomerulonephritis
- KIDNEYS –> leads to necrotizing hemorrhagic interstitial pneumonitis
- is an auto-immune disorder: body makes Ab against type IV collagen a3 chain which is in the
- demo: 20 yr old male smokers
goodpasture - gross morphology
heavy lungs with red-brown consolidation
goodpasture - microscopic morphology
- intra-alveolar RBCs + hemosidrin stained macrophages (like in all diffuse pulmonary hemorrhage syndromes)
- can also have:
- septal fibrosis
- type II pneumocyte hypertrophy*
- l_inear IgG_ on septal basement membrane
- capillaritis (PMNs in capillaries)
goodpastures clinical presentation
- LUNGS: hemoptysis / necrotizing hemorrhagic interistitial pneumonitis
- KIDNEYS: glomerularnephritis –> rapid renal failure
idiopathic pulmonary hemosiderosis
- definition
- pathogenesis
- demographics
- type of diffuse pulmonary hemorrhage syndrome
- unknown cause
- demo: children** mostly
idiopathic pulmonary hemosiderosis - microsocpic morphology
- similar to all diffuse pulmonary hemorrhagic syndromes
- intra-alveolar RBCS + hemosiderin laden macrophages
polyangitis with granulomatosis (wegeners)
- definition
- pathogenesis
- a type of diffuse hemorrhagic syndrome
- autoimmune disease: T-cell mediated hypersensitivity to benign inhaled agents, effecting:
- LUNG:
- necrotizing granulomas of URT/LRT
- necroziting vasculitis - small/medium vessels
- KIDNEYL
- focal necrotizing glomerulonephritis
- LUNG:
- autoimmune disease: T-cell mediated hypersensitivity to benign inhaled agents, effecting:
wegeners granuomatosis - morphology
gross/microscopic
- granulomas of geographic pattern with central necrosis + associated vasculitis –> see in UCERS in the nose/palate/pharynx
- large cavititazing lesions –> LUNG
dx of wegners (granulomatosis with polyangitis)
PR3-ANCA (C-ANCA)
contrast the kidney manifestations of wegners vs goodpastures
wegners: focal necrotizing glomularnephritis
goodpastures: rapid progressive glomerularnephritis
