Path Flashcards
histology of respiratory tree
ciliated pseudostratified columnar epithelium
cartilaginous airways with mucus secreting goblet cells and submucosal glands
alveolar histology
capillary endothelium basement membrane intersititium alveolar epithelium alveolar macrophages
dual circulation
pulmonary and bronchial arteries
pulmonary tract defense mechanisms
nasal clearance
tracheobronchial clearance by cilia
alveolar clearance by macrophage
factors that affect pulmonary defense mechanisms
loss of cough reflex (coma, drugs, pain)
injury to mucociliary apparatus (ciliary dysfunction, smoking, viruses)
inerference with alveolar macrophages (alcohol, smoking)
pulmonary congestion and edema
accumulation of lung secretions (CF)
presentation of interstitial diseases
dyspnea, tachypnea, cyanosis without wheezing
reduced DLCO, reduced lung volume, reduced compliance
chest x-ray of interstitial diseases
small nodules, irregular lines or ground glass shadows
pathogenesis of idiopathic pulmonary fibrosis
inflammation causes abnormal epithelial repair leading to fibroblastic/myofibroblastic proliferation (fibroblastic foci)
TGF-beta1 released by type 1 alveolar epithelial cells-apoptosis
caveolin-1 which inhibits TGF-beta1 is decreased
morphology of IPF
cobblestone pleural surface
mostly lower lobes
temporal heterogeneity
later-dense fibrosis
clinical course of IPF
insidious onset of dyspnea on exertion and dry cough
hyoxemia, cyanosis, and clubbing
mean survival 3 years
morphology nonspecific interstitial pneumonia
cellular-uniform/patchy inflammation
fibrosing pattern-interstitial fibrosis without temporal heterogeneity, no fibroblastic foci, no honeycomb
clinical course of nonspecific interstitial pneumonia
dyspne and cough
better prognosis than UIP
etiology of cryptogeneic organizing pneumonia
response to various infections or inflammatory lung injury
morphology of cryptogenic organizing pneumonia
subpleural or peribronchial airspace consoidation
no temporal heterogeneity
no interstitial fibrosis or honeycomb lung
Masson bodies
intra-alveolar polypoid plugs of loose organizing connective tissue
seen in cryptogenic organizing pneumonia
clinical course of cryptogenic organizing pneumonia
acute onset of cough and dypsnea
steroids for >6 months
pulmonary involvement in connective tissue diseases
RA, sceroderma, and SLE
can occur in different patterns
pathogenesis pneumoconioses
depends on physical factors
most dangerous particles 1-5 um
asymptomatic anthracosis
accumulation of carbon pigment in macrophages in miners, tobacco smokers, and urban dwellers
does not produce a cellular reaction
simple coal workers pneumoconiosis
coal macules along the respiratory bronchioles
upper lobes and upper zones of lower lobes
leads to centrilobular emphysema
complicated coal workers pneumoconiosis
multiple blackened scars>2cm in lung parenchyma
dense collagen, carbon pigment and necrotic centers
clinical course of coal workers pneumoconiosis
mild forms of complicated can exist without loss of function
more advanced can lead to HTN or cor pulmonale
most prevalent chronic occupational disease worldwide
silicosis
pathogenesis of silicosis
silica in crystalline and amorphous forms
crystalline more fibrogenic
causes macrophage to release mediators
morphology silicosis
early-nodules in upper lung zones
later coalescing to from hard collagenous scars of concentric layers of hyalinized collagen with dense capsule
involve hilar lymph nodes and pleura with thin calcifications (eggshell calcifications)
silicosis clinical course
x ray shows fine nodularity in upper lung areas
dyspnea occurs after development of massive fibrosis
associated with increased incidence of TB
pathogenesis asbestos
depends on concentration
fibrogenic forms-serpentine and amphibole
fibrous pleural plaques, pleural effusions, interstitial fibrosis, lung and laryngeal carcinoma, mesothelioma, colon carcinoma
injury at bifurcations of small airways-penetrate macrophage
serpentine from
most prevalent
curly and flexible
removed by upper respiratory mucociliary apparatus
amphibole form
least prevalent
stiff and brittle fibers
penetrate deep into interstitium
associated with mesothelioma
morphology of asbestos exposure
interstitial fibrosis similar to UIP
interstitial fibrosis around respiratory bronchioles and alveolar ducts
honeycomb pattern-begins in lower lobes
pleural plaques
components of pleural plaques
dense collagen with calcium mostly on AP parietal pleura and diaphragm
do not contain asbestos bodies
asbestos bodies
golden brown elongated asbstos fibers coated with iron-containing protein from macrophage
therapy induced lung disease
bleomycin
amiodarone
acute radiation pneumonitis
chronic radiation pneumonitis
Caplan syndrome
coal worker pneumoconiosis and RA
nodules with possible central necrosis
epidemiology of sarcoidosis
higher prevalence in women, particularly AA and SE US
diagnosis of exclusion
pathogenesis of sarcoidosis
immunologic-increased CD4, IL2, IL8, TNF
anergy to certain skin test antigens (candida, PPD)
morphology of sarcoidosis
noncaseating granulomas with epithelioid cells, giant cells, Schaumann bodies, asteroid bodies
lung findings sarcoidosis
granulomas along lymphatics, bronchi, blood vessels
lymph nodes in sarcoidosis
hilar and mediastinal nodes
liver in sarcoidosis
granulomas in portal tracts
skin and mucous membranes in sarcoidosis
nodules, plaques, flat lesions
eyes and salivary glands in sarcoidosis
iritis with lacrimal gland infalmmation (dacroadenitis)
mikulicz syndrome
dacroadenitis+bilateral involvment of parotic/submax/sublingual
best prognosis sarcoidosis
hilar adenopathy alone
worst prognosis sarcoidosis
pulmonary involvement alonge
hypersensitivity pneumonitis
immunologically mediated
due to increased reactivity to antigen
can by type III or type IV hypersensitivity
farmers lung
spores of actinomycetes
pigeon breeders lung
proteins from feathers/excretions
humidifer lung
bacteria from heated water reservoir
morphology hypersensitivity
chronic interstitial inflammation
noncaseating granulomas
interstitial fibrosis and honeycombing
xray from hypersensitvity
diffuse and nodular infiltrates
time frame for acute attacks
sensitized patients with fever, dyspnea, cough, leukocytosis
4-6 hours after antigen exposure
chronic attacks of hypersensitivity
progressive respiratory failures and decreased lung capacity and compliance
restrictive
acute eosinophilic pneumonia
rapid onset
BAL>25% eosinophils
good response to corticosteroids
simple pulmonary eosinophilia (Loeffler’s syndrome)
transient lung lesions with blood eosinophilia
CXR shows irregular lung densities
thickened alveolar septa containing eosinophils and giant cells
secondary pulmonary eosinophilia
parasitic, bacterial, and fungal infections
associated with asthma, allergic bronchopulmonary aspergillosis and drug allergies
chronic eosinophilic pneumonia
areas of lung consolidation at the lung periphery
eosinophils and lymphocytes in septal wall and alveolar spaces
high fever, night sweats and dyspnea
treated with steroid therapy
morphology of desquamative interstitial pneumonitis
large numbers of inta-alveolar macrophage containing brown pigment (smokers macrophages)
thickened alveolar speta with sparse inflammation
clinical desquamative interstitial pneumonitis
more common in men around 40s-50s
almost all smoke
clubbing of digits
response to steroid therapy and cessation of smoking
respiratory bronchiolitis associated interstitial lung disease morphology
large smokers macrophage numbers within respiratory bronchioles, alveolar ducts, peribronchiolar spaces
patchy chronic submucosal and peribronchiolar inflammation
mild peribronchiolar fibrosis with centrilobular emphysema
etiology of acquired pulmonary alveolar proteinosis
anti-GM-CSF antibodies
impaired clearance of surfactant
etiology of congenital PAP
mutations
secondary PAP etiology
secondary to silicosis, immunodeficiency and malignancies
morphology of PAP
heavy lungs
dense intra-alveolar granular precipitate that is PAS positive and contains cholesterol clefts
minimal inflammatory infiltrate
clinical PAP
mostly in adults with productive cough containing chunks of gelatinous material
most common cause of cancer mortality
lung cancer
genetics of squamous cell carcinoma
3p, 9p (CDKN2A), 17p (TP53) deletions
loss of Rb
amplification of FGFR1
genetics of small cell carcinoma
loss of TP3, RB
3p deletions
MYC amplifications
genetics of adenocarcinoma
GoF in receptor tyrosine kinase
KRAS mutations
lung cancers in non-smokers
most have EGFR
women, adenocarcinoma
precursor lesions
squamous dysplasia
atypical adenomatous hyperplasia
adenocarcinoma in situ
neuroendocrine cell hyperplasia
charactersistics of atypical adenomatous hyperplasia
less than 5mm, dysplasic pneumocytes lining alveoli
characteristics of adenocarcinoma in situ
less than 3cm
dysplastic cells lining alveolar septae with no invasion
characteristics of hamartoma
coin lesion on radiology
solitary, well circumscribed
epithelial and stromal component
characteristics of lymphangioleiomyomatosis
young child bearing age owmen
proliferation of perivascular epithelaioid cells, melanocytes, smooth muscle markers
genetics lymphangioleiomyomatosis
loss of function TSC2
treatment lymphangioleiomyomatosis
transplant
characteristics of inflammatory myofibroblastic tumor
children, single, well defined mass
fibroblasts, myofibroblasts, lymphs, plasma cells
genetics of myofibroblastic tumor
rearrangement of ALK
location of squamous cell carcinoma
centrally located
morphology squamous cell carcinoma
large mass, necrosis
keratin pearls
intercellular bridges
p63 and p40 expression
carcinoid tumor histology
no necrosis
less than 2 mitoses/10HPF
atypical carcinoid tumor histology
2-10 mitoses/10HPT
may have focal necrosis
paraneoplastic syndromes
SIADH Cushing syndrome hypercalcemia/hypocalcemia gynecomastia carcinoid syndrome
benign pleural tumors
solitary fibrous tumor
kinds of malignant mesothelioma
epithelioid
sarcomatoid
mixed
