MHD Flashcards
(117 cards)
1
Q
What is the relationship between Calveolin and Idiopathic Pulmonary Fibrosis?
A
Epithelial activation/injury leads to the release of TGF-B1 and this goes to block Caveolin in the fibroblasts. Caveolin normally functions to block deposition of collagen and extracellular matrix. W/o Caveolin, pulmonary fibrosis has a clearer path to development.
2
Q
Anthracosis
A
Deposition of coal dust in the lungs
One form of Pneumoconioses (other examples include silicosis and asbestosis)
3
Q
What is the key characteristic of sarcoidosis?
A
Non-caseating granulomas
4
Q
What are Schaumann bodies and Asteroid Bodies, and what disease process are they associated with?
A
Schaumann: Concretions (hard masses) of calcium and protein
Asteroid: Star like stellate inclusions
Both found in SARCOIDOSIS
5
Q
What changes in FEV1 w/ a bronchodilator defines reversibility?
A
12% and 200cc’s
6
Q
99% of obstructions are _________ (upper/lower) airway.
A
Lower
7
Q
Equation for Diffusion Capacity
A
DLCO = [CO]inhaled - [CO]exhaled
This is the difference between the CO inhaled and the CO exhaled, which tells you how much ended up diffusing into the blood.
8
Q
If DLCO is low, but spirometry and lung volumes are normal, what lung issue might you suspect?
A
Pulmonary HTN
9
Q
What are the (3) main components to a PFT and what do they each tell you?
A
- Spirometry (used to ID obstruction)
- Long Volume Determination (used to ID restriction)
- Diffusion Capcity Measurement (used to ID diffusion defect)
10
Q
What are the (4) Categories for Asthma and what values define each (based on each criteria below):
*daytime symptoms/wk, nighttime symptoms/mo, FEV1 or PEFR, PEFR variability
A
11
Q
RADS
A
Reactive Airway Dysfunction Syndrome
No prior asthma, then a “big-bang” exposure (ex. 9/11) leads to asthma which normally resolves in 6 months or so.
12
Q
Samter’s Triad
A
A triad of symptoms which point to aspirin sensitivity:
- Asthma
- Nasal polyposis
- NSAID sensitivity
13
Q
Allergic Bronchopulmonary Aspergillous
A
Asthma + Allergic response (+ skin test, IgE, eosinophils, etc.)
14
Q
Stages of Sarcoidosis based on CXR
A
LAD = lymphadenopathy
ILD = Interstitial lung disease
15
Q
What are your (3) main differentials when a pt presents w/ a cough?
A
- Postnasal drip
- Acid reflux
- Asthma
16
Q
Fleischner Guidlines
What is it used for, and what are the guidlines?
A
Used for determining course of action after nodules found on low dose CT scan.
17
Q
Identify the type of lung cancers being shown in each section
A
- Squamous cell
- Adenocarcinoma
- Small cell carcinoma
- Large cell undifferentiated carcinoma
18
Q
Which bug is the most common cause for Community Acquired Pneumonia?
What are the clinical presentations associated with this bug (5)?
What does this bug look like?
A
Strep Pneumo
Acute onset fever, shaking chill, rusty sputum, shortness of breath, pleuritic chest pain
Lancet shaped diplococcus
19
Q
ALL
Name the cytogenic markers which correlate with a good prognosis.
Name the cytogenic markers which correlate with a poor prognosis.
A
Good sign:
- t(12;21) [TEL1-AML1]
- Hyperdiploidy
Poor signs:
- t(9;22) [BCR-ABL] - Philidelphia chromosome
- t(4;11) [AF4-MLL]
20
Q
Name the 4 WHO classifications of AML
What are the affliated cytogenic markers and what do they indicate?
A
- AML w/ recurrent genetic abnormalities
- t(15;17)PML/RARA good prognosis
- t(11q23;v)MLL poor prognosis
- AML arising from Myelodysplastic Syndrome (MDS) poor prognosis
- Therapy Related poor prognosis
- post chemo/radiation
- NOS intermediate prognosis
21
Q
What is Myelodysplastic syndrome? What can it progress to?
A
It is also known as “preleukemia”
Hypercellular bone marrow which leads to a peripheral cytopenia and many morphological abnormalities. Often progresses to AML.
Poor prognosis
22
Q
Acute Promyelocytic Anemia
What is it a type of? What is the genetic change? What does it increase risk of? How is it treated? What is the prognosis?
A
It is one of the classifications of AML
t(15;17)PML/RARA
There is fusion between the PML/Retinoic Acid Receptor genes creating an abnormal RAR.
Greatly increased risk of DIC due to the presence of MPO based auer rods.
Great prognosis if treated with all-trans retinoic acid (ATRA)
23
Q
What is SIRS and what qualifications must be present for something to count as SIRS? (4 total options)
A
Systemic Inflammatory Response Syndrome
Massive inflammatory reaction from systemic cytokine release
At least 2 of the following must be present:
- Temperature >38° C or
- Heart rate >90
- Respiratory rate >20 or PaCO2
- WBC >12,000 or 10% immature
24
Q
What are the (2) key cytokines in Sepsis Syndrome?
What 3rd cytokine has a key role?
A
TNFa and IL-1
IL-6 also plays a key role
25
List the (3) components of clinical shock
1. **Systemic arterial Hypotension**
2. **Clinical signs of hypoperfusion**
* _Skin_ (cold, clammy, etc.)
* _Renal_ (oliguria)
* _Neurologic_ (AMS)
3. **Hyperlactemia** (due to decreased muscle perfusion)
26
What should be completed in first (3) hours of Sepsis Bundle? (4)
**First (3) Hours:**
1. Measure lactate level
2. Obtain blood cultures prior to administration of antibiotics
3. Administer broad spectrum antibiotics
4. Adminster 30 mL/kg crystalloid for hypotension or lactate \> or equal to 4mmol/L
27
What should be completed within (6) hours of the Sepsis Bundle? (3)
1. Apply vasopressor if it doesn't respond to fluid resuscitation (to maintain MAP over 65 mmHg)
2. Measure CVP and CV O2 sats if hypotension persists or initial lactate was high
3. Remeasure lactate if initial lactate was elevated
28
When markers/factors correlate with a better prognosis for CLL/SLL? Worse?
_Better_: Mutations of the IGH gene
_Worse_: Richter syndrome/transformation to a higher process
29
Cytochemical staining for Hairy Cell Leukemia
Tartrate resistant acid phosphate (TRAP)
30
What is the cause for Adult T-cell Leukemia/Lymphoma? What kind of cells does it lead to and what is the prognosis?
HTLV-1
Usually fatal and leads to floret-like CD-4 cells
31
What is the key genetic change which leads to Follicular Lymphoma?
A t(14;18) BCL2/IGH fusion which leads to BCL2 over-amplification and an inability for apoptosis to occur as a result.
32
Key B cell markers?
T cell markers?
Myeloid markers?
B: CD19, CD20
T: CD3
Myeloid: Myeloperoxidase
33
Key genetic change in Mantle Cell Lymphoma
t (11;14) [Cyclin D1-IGH fusion] leading to cyclin D1 over-amplification and increased proliferation as a result.
34
Key translocations for Burkitt lymphoma
t(8;14) [MYC/IGH fusion]
Leads to MYC over-amplification and increased proliferation as a result
35
What are the 2 distinct subtypes of Diffuse Large B-cell lymphoma? What are the potential cytogenic features associated with diffuse large B-cell lymphoma?
**Subtypes**:
1. Germinal Center B-cell (GCB): better prognosis
2. Activated B-cell (ABC)
**Cytogenic features**:
* t(14;18)
* BCL6
* double hit: t(14; 18) + BCL6 (_very poor prognosis_)
36
What is the cytological change associated with CML? What does this lead to? What does the BM look like? How are the progentitors affected?
BCR-ABL fusion (philadelphia)
Early transformation leads to B and T cell involvement. This leads to a marked increase in neoplastic granulocytic precursors in the marrow.
Close to 100% BM cellularity
More megakaryocytes, fewer erythroid progenitors, and **High basophil count**
37
What is the mutation which leads to polycythemia vera?
JAK2 mutations
38
What are the mutations which lead to primary myelofibrosis (chronic idiopathic myelofibrosis)? What is the pathogenesis of this disease?
JAK2, CALR, and MPL point mutations
This disease is an early progression to obliterating marrow fibrosis. There are excessive collagen deposits in bone marrow due to secretion of **PDGF** and **TGF-B** by _neoplastic megakaryocytes_.
Normal marrow elements are displaced.
39
What are the mutations which cause essential thrombocytosis? What happens and what is the treatment.
Activating JAK2/MPL mutations. These lead to proliferation of the megakaryocytic lineage . Treated with aspirin.
40
Name the (3) Phases of ARDS
1. _Acute_- early **exudative** phase
2. _Organizing_- subacute **proliferative** phase
3. _Late_- **Fibrotic** phase
41
Acute interstitial pneumonia
A catch-all term for a disease which presents like ARDS w/o any known etiology (thus ruling out ARDS). All declared after everything else has been ruled out.
Average time until patient dies = 2 months
42
What (2) diseases fall under the umbrella of COPD?
Emphysema and Chronic Bronchitis
43
Atopic vs Non-atopic Asthma
_Atopic_: Immune-mediated. Involves IgE and mast cells. Triggered by environmental allergens. + skin test.
_Non-atopic_: Non-immune triggering mechanisms (Hyperirritable bronchial tree; virus induced inflammation of the respiratory mucosa, etc.). - skin test
44
Name (2) of the key genes involved in asthma
1. Chromosome 5q (has several susceptibility genes)
2. ADAM 33-20q
45
(3) key histological findings associated with asthma
1. Curshmann spirals (mucous plugs + whirls of shed epithelium)
2. Eosinophils
3. Charcot-leyden Crystals
46
Describe the pathophyiology of Chronic Bronchitis
What is the reid index and how does it factor in?
Hypertrophy of mucus secreting glands (goblet cell metaplasia). Leads to inflammation and fibrosis. Could result in cor pulmonale, infections, or carcinoma.
**Reid index**: gland:wall ratio. It is increased in CB.
Blue Bloater
47
Centriacinar emphysema
Where is it in the lung? What is the usual patient demographic? What other disease is it oft associated with?
C.R.U.M.
Coal workers/ Chronic Bronchitis
Respiratory Bronchioles
Upper lobes
Male Smokers
48
Panacinar emphysema
What parts of the lung are affected? What genetic disorder is oft associated?
Lower lobes/ whole acinus
Alpha-1-antitrypsin deficiency
49
Distal acinar emphysema
What part of the lung is affected? What does the tissue look like as a result?
Distal acinus (alveolar ducts/alveoli)
Adacent to areas of fibrosis, scarring or atelectasis. Can form huge cyst **(bullae)**
50
Irregular emphysema
What tissues are involved and what do they look like? Describe the clinical manifestations.
Acini are irregularly involved and have scarring/healed inflammatory lesions.
_Mostly asymptomatic_
51
What is the normal phenotype for the alpha 1 antitrypsin proteinase inhibitor? What is the common phenotype in deficiency?
_Normal_: *PiMM*
_Deficiency_: *PiZZ*
52
What conditions are predisposing for Bronchiectasis? Describe the basic pathogenesis.
**O.H.I.O.**
_Obstruction_
_Hereditary_ (CF, Kartagener's syndrome, Immunodeficiency)
_Infection_
_Other_ (RA, SLE, GvHD)
Pooling of nasty stuff due to impaired cleaning mechanisms, which leads to inflammation, necrosis, fibrosis and eventual dilatation with foul-smelling/purulent sputum
53
What chromosome is defected in Cystic Fibrosis?
Chromosome 7 (CFTR gene)
54
Kartagenor Syndrome
Malformed, non-functional cilia due to absence or shortening of dynein arms. Leads to:
1. sinusitis
2. bronchiectasis
3. situs inversus
4. infertility
55
Describe the pathogenesis of _Idiopathic Pulmonary Fibrosis_
1. Repeated cycles of epithelial activation/injury by some unidentified agent
2. Abnormal epithelial repair
3. Fibroblastic proliferation
4. Release of TGF-B due to destruction also blocks **Caveolin** which is normally responsible for blocking collagen and extracellular matrix deposits.
56
What do the lungs look like, for patients with Idiopathic Pulmonary Fibrosis?
* Cobblestone lung due to irregular scarring
* Rubbery surface
* Can be honeycombing
57
What is the name for inhaled carbon pigment? How does this lead to fibrosous?
**Anthracosis**
This and other deposited entities in pneumoconioss are eaten by macrophages, leading to the release of inflammatory mediators and subsequent fibrogenesis and collagen deposits.
58
Caplan Syndrome
**Rheumatoid Pneumoconiosis**
Coal deposits (anthrocosis) + RA
Nodules may cavitate in radiology
59
What is characteristic about the nodules in silicosis? How does the lung look when silicosis is advanced?
Nodules w/ concentric hyalinized collagen. Nodules often coalescence, leading to progresive massive fibrosis.
You get a carred, contracted upper lobe in advanced silicosis.
60
What is another name for an asbestos body?
Ferruginous body
61
Smoking adds how much of a risk of lung cancer to someone who also has asbestos exposure? How much of an increased risk of malignant mesothelioma is there for a smoker?
55x
62
How is sarcoidosis dx?
Dx based on a transbronchial biopsy. It is a diagnosis of _exclusion_, characterized by **non-caseating granulomas**.
63
What FEV1 percent values correlate with...
1. Mild severity?
2. Moderate severity?
3. Severe severity?
1. Mild: 80-60%
2. Moderate: 60-40%
3. Severe:
64
Tram tracking and the signet ring sign are associated with which disease process?
Bronchiectasis
65
Key tennets for COPD treatment (5)
1. STOP SMOKING
2. Medications
3. Long-term Oxygen Therapy
4. Cardiopulmonary Rehab
5. Other (transplant)
66
Idiopathic Pulmonary FIbrosis Rx
1. Pirfenidone
2. Nintedanib
67
2 main causes for central sleep apnea
CNS disease or _heart failure_
68
Mallampti Classification is based on...
Space between the tongue and the Uvula.
Used for determination of obstructive sleep apnea
69
What are Loculations? And what do they imply?
One sign associated with pleural effusion.
Thick walled off lung area housing fluids. Implies adhesions and indicates infections. Antibiotics + surgery.
70
What is the max fluid amount that can be removed from a P. Effusion at a time? What is this process called?
_Thoracentesis_- max of 1.5L removal
71
Define the (4) Light's Criteria for Exudates
TPpl/TPserum \>0.5
LDHpl/LDHserum \>0.6
LDHpl \>200
Cholesterolpl \>45
If any one of these is present, the fluid is exudate. Otherwise it is transudate.
72
Empyema
Infection of plural space
73
Pleurodesis
Removal of pleural space via chemical or mechanical irritiation
74
1% change in body weight will give you a \_\_\_% change inthe apnea-hypopnea index (AHI)
3%
75
In addition to hypersomnolence and hypoxia, what is another major consequence of sleep apnea?
Pulmonary HTN
76
Lead-time bias vs Length-time bias
_Lead_: Pt knows they have cancer earlier but die at same point anyway
_Length_: More cancers are found and cured but that increase is due to slow growing cancers that weren't going to kill the patient anyway
77
FInding of a _non_-calcified nodule in the lung is a ________ (good/bad) sign
Bad
78
USPSTF guidlines for lung cancer screening
_annual_ screening for adults _55-80_ w/ _30 pack-year history_ and who smoked in last _15 years_
79
Keritization and intracellular bridges are associated with which cancer type?
Squamous cell carcinoma
80
What type of immuno-reactivity does SCC how? What kind of mutations are often present in SCC?
* Shows p63/p40 immuno-reactivity
* p53 mutations are most common.
81
What criteria must be present for a dx of adenocarcinoma?
1. Gland forming, or
2. Mucin forming, or
3. + TTF-1
82
Describe the progression of the adenocarcinoma precursors
AAH [AIS/BAC] in MIA, now I have [ADC]
**AAH**- well demarcated; less than 5mm; cuboidal/low columnar; mild atypia
**AIS/BAC**- less than 3cm; preservation of alveolar architecture; no invasion
**MIA**- 3cm or less and invasive focus (less than 5mm); no pleura invasion, lymphatics, blood vessels
83
What are the (3) activating mutations for ADC? Describe key features for each
1. **EGFR-** always active tyrosine kinase; asian women; Erlotinib treatment
2. **ALK-** younger patients; non-smokers; Crizotinib treatment
3. **KRAS-** 30% caucasion; worse outcomes; no specific targeted therapy
84
Inactivation of what (2) genes is present in 100% of small cell carcinoma (oat cell carcinoma)?
p53 inactivation
RB inactivation
85
Bronchial Carcinoid
Tumor type? Invasion/Metastatis? Prognosis?
* Low grade malignant neuroendocrine tumor
* Locally invasive/ rarely metastatic
* Good prognosis
86
Chylothorax
Lymphatic fluid in the pleural space
87
What are the key complications of lung cancer?
SPHERE
* Superior vena cava syndrome
* Pancoast tumor
* Horner syndrome
* Endocrine (paraneoplastic)
* Recurrent laryngeal nerve compression
* Effusions (pleural or pericardial)
88
Kerly B-lines on X-ray are indicative of what disorder?
High Pressure Pulmonary Edema
89
What is the gold standard of PE dx?
Pulmonary angiogram
90
What vasodilator is given for Pulmonary HTN?
CCBs, and NO OTHER VASODILATORS
91
What are the common bugs for CAP? What are the atypical CAP bugs?
_Common_: S. Pneumo, H. Inf., Moraxella catarhalis, Staph aureus
_Atypical_: Legionella, Mycoplasma, Chlamydia + viruses
92
Pharyngitis usually has a ______ etiology
Viral
93
Classic presentation for S. Pneumo
1. Rusty sputum
2. Pleuritic chest pain
3. Fever
4. Shaking chill
5. SOB
94
Therapy for influenza
-Mivir drugs
95
CAP in Pediatrics
What bug is expected for a child under 2?
5-10?
10-16?
5-10 = mycoplasma
10-16= S. pneumo; chlamydophilia
96
**Therapy of CAP:**
Outpatient?
Inpatient?
_Outpatient_: Macrolide/Doxycyline; Resp. fluoro if co-morbid illness/recent antibiotic
_Inpatient, ICU or non-ICU_: resp. Fluro (moxifloxacin); B lactam + macrolide
97
Therapy of CAP + Pseudomonas
Anti-pseudomonal B lactam +respiratory fluoroquinolone
or
Anti-pseudomonal B lactam + macrolide + aminoglycoside
98
Chronic Pneumonia Bugs
MEAN CAT
1. Mycobacterium
2. Endemic Fungi
3. Actinomyces
4. Nocardia
5. Coxiella
6. Anatomic Problem
7. Tularemia
99
CLL/SLL and transformation
Some patients transform to DLBL or prolymphocytic leukemia
100
What kind of organisms is a patient susceptible to if they have Neutrophil dysfunction/deficiency?
1. Gram negative rods
2. Staph
3. Aspergillus
4. Candida
101
What kind of organisms is a patient susceptible to if they have T cell dysfxn or deficiency?
1. Fungi
2. Viruses
3. Pneumocystis
4. Mycobacteria
5. Listeria
102
What kind of organisms is a patient susceptible to if they have B cell deficiency?
Encapsulated
103
What characteristics of TB are not present in Nontuberculous Mycobacteria? What is the main predisposition for non-TB myco? How does it spread?
* It spreads via environmental pathogens (non-cough bioaerosols; ater; soil; nosocomial)
* Main predisposition= Structural Lung Abnormality
* _NO_ latent phase; _NO_ dissemination; _NO_ skin test/QG
104
What tests are used for confirmation of TB after PPD/QG and CXR?
1. Acid fast bacillus of sputum, or
2. PCR, or
3. Molecular tests, or
4. Liquid media culture
105
Severe sepsis vs Septic shock
_Severe sepsis:_ Sepsis + Organ dysfunction\*/ hypoperfusion
_Septic shock_: Sepsis + Refractory hypotension + hypotension abnormalities
\*Organ dysfunction= H.O.L.A.
* Hypoxemia
* Oliguria
* Lactic Acidosis
* AMS
106
When is hydrocortisone given for patients with sepsis?
Only when the hypotension is refractory
107
Staining for Candidiasis
+ Gram stain or calcofluor
PAS
Test for germ tube presence
108
Key to ID of Aspergillus fumigatus
ID according to color and structures
109
Mucormycosis Diagnosis
Microscopic exam of tissues for broad, non septated hyphae
Growth is wooly white to grey
110
Name the (3) genera of dermatophytes. How do you determine which is which?
_EMT_
1. Epidermophyton
2. Microsporum
3. Trichophyton
To determine which is which, scrape at the leading edge and examine via KOH or Calcofluor. Can also use wood's light. Can also id based on conidia shape.
111
Where is Cryptococcus neoformans found
Throughout the world
112
Where is blastomycosis found? What do the buds look like for this fungus?
Ohio river valley + great lakes + south east coast
Broad Based Buds
113
Where is histoplasmosis found?
Ohio/mississippi river valley
114
Where is Cryptococcus gotti found?
West coast (crips on the westside)
115
Where is Coccidioidomycosis found?
Southwest
116
What is the diagnostic method of choice for Cryptococcal infections? What are other useful techniques (4)?
Latex agglutination of the cryptococcal antigen is gold standard.
India pink prep, GMS, Gram stain, and mucicarmine are also very useful.
117
What form does Coccidioides immitis take in the lung tissue? In culture?
_Tissue_: spherules filled with endospores
_Culture_: mold w/ barrel shaped anthoconidia
