Robbins and rosai Flashcards
Histologic manifestation of Acute lung injury(ALI)/acute respiratory distress syndrome(ARDS)
Diffuse alveolar damage
Appearance of acute lung injury in the absence of known triggers and follows a rapid progressive clinical cause
Acute interstitial pneumonia
4 events in the pathogenesis of diffuse alveolar damage(dad)
- Endothelial activation
- adhesion and extravasation of neutrophils
- accumulation of intra-alveolar fluid and formation of hyaline membranes
- resolution of injury
Characteristically lines the alveolar walls in DAD
Hyaline membranes
congenital lesion characterized by the presence of variously sized intercommunicating cysts lined by cuboidal-to-ciliated pseudostratified columnar (‘adenomatoid’) epithelium
Congenital cystic adenomatoid malformation (CCAM)
refers to the permanent dilation of bronchi usually associated with destruction of some elements of the bronchial wall and inflammatory changes that extend into the surrounding or distal lung parenchyma.
Bronchiectasis
a condition characterized by fixed radiological abnormalities limited to the right middle lobe and/or lingula
Middle lobe syndrome
autosomal recessive disease causing mutations result in ciliary dysfunction due to defects in ciliary motor proteins preventing mucociliary clearance, setting the stage for recurrent infections that lead to bronchiectasis
Primary ciliary dyskinesia
hallmark of the disease is a compact non- caseating granuloma composed of epithelioid cells, Langhans giant cells, and lymphocytes
Sarcoidosis
most common of the idiopathic interstitial pneumonias and has marked regional variation in the nature and degree of the fibrosis, with a distinct patchwork distribution and architectural derangement in the form of scarring and honeycomb change
Usual interstitial pneumonia (UIP)
A smoking related diffuse lung disease characterized by an accumulation of lightly pigmented alveolar macrophages within respiratory bronchioles spilling into neighboring alveoli
respiratory bronchiolitis- interstitial lung disease (RBILD)
a smoking related, mild, paucicellular fibrosis in the form of lamellar eosinophilic collagenous thickening of alveolar septa in a patchy, mainly subpleural distribution
smoking- related interstitial fibrosis (SRIF)
second most common finding in patients with interstitial pneumonias of unknown cause. findings may be patchy or diffuse but retain a degree of qualitative uniformity in areas of abnormality
nonspecific interstitial pneumonia (NSIP)
a rapidly progressive form of idiopathic interstitial pneumonia and is synonymous with the Hamman–Rich syndrome
Acute interstitial pneumonia (AIP)
represented by fibroblastic plugs (‘Masson bodies’) filling air spaces
organizing pneumonia
acute lung injury secondary to nitrogen dioxide inhalation and is characterized by the presence of DAD without granulomatous inflammation
Silo-filler’s disease
T/F Langerhans Cell Histiocytosis occurs almost exclusively in cigarette smokers
True
Immunohistochemically Langerhans cells can be highlighted by (give 3)
S-100, CD1a, or langerin
mutation associated with LCH
BRAFV600E mutations
histiocytic disorder which can affect the lung, in conjunction with bones, soft tissues, and the central nervous system characterized by an infiltrate of foamy histiocytes, lymphocytes, and Touton giant cells
Erdheim–Chester disease
characterized by silica associated micronodular scars with a characteristic pattern of central lamellar fibrosis distributed in a bronchiolocentric pattern with a cellular periphery in which dust-laden macrophages predominate
Silicosis
non-neoplastic reaction of the lungs to inhaled mineral or organic dust
Pneumoconiosis