Step Review 2: Musculo, Respiratory, Renal, Hem Onc Flashcards
1
Q
Which parts of the femur do the ACL and PCL originate from?
A
ACL: lateral condyle
PCL: medial condyle
2
Q
What is the unhappy triad? Why is it slightly misleading?
A
ACL, MCL and medial meniscus tear. The lateral meniscus is more commonly injured
3
Q
What bursa gets inflamed from excessive kneeling?
A
Prepatellar bursa (e.g. tile layer)
4
Q
What bursa does a Baker cyst involve?
A
Gastrocnemius/semimembranosus bursa
5
Q
Name the rotator cuff muscles and their innervations
A
Supraspinatus: suprascapular nerve
Infraspinatus: suprascapular nerve
teres minor: axillary nerve
Subscapularis: upper and lower subscapular nerves
6
Q
Which is closer to the thumb: scaphoid or trapezium?
A
Trapezium
7
Q
Between the trapezium, trapezoid, and triquetrum, which is in the proximal layer of wrist bones and which in the distal layer is most lateral?
A
Triquetrum is proximal (“try), trapezium is more lateral (thumb side)
8
Q
Dislocation of which wrist bone may cause carpal tunnel syndrome? What might cause bilateral carpal tunnel?
A
Lunate, dialysis-related amyloidosis
9
Q
Is sensation compromised in carpal tunnel?
A
No, the palmar cutaneous branch enters the hand external to the carpal tunnel.
10
Q
Which ligament is cut to relieve carpal tunnel syndrome?
A
Transverse carpal ligament
11
Q
What nerve does sensation over the lateral arm? Lateral forearm?
A
Lateral arm= axillary
Lateral forearm= musculocutaneous
12
Q
What specific branch of the median nerve supplies the thumb?
A
The recurrent branch
13
Q
What nerve supplies sensation to the posterior, medial hand?
A
Ulnar nerve, radial only does the radial side (e.g. opposite of median)
14
Q
On the posterior hand, does the radial or median nerve do the tips of the thumb side fingers?
A
Median
15
Q
Which nerve causes a winged scapula?
A
Long thoracic nerve (C5-C7)
16
Q
What is klumpke palsy? How does an adult get it typically?
A
Total claw hand due to traction/tear of lower trunk of brachial plexus. Typically grabbing a tree branch on the way by or something similar
17
Q
What is the action of lumbricals?
A
Extend DIP, PIP and flex MCP
18
Q
What is compressed in thoracic outlet syndrome? Where?
A
Lower trunk of brachial plexus and subclavian vessels (scalene triangle)
19
Q
What nerve might be injured if the arm cannot be abducted above the horizontal position?
A
Long thoracic nerve, serratus anterior, winged scapula
20
Q
Injury when you see foot drop and loss of sensation on dorsum of foot? Where is the injury likely?
A
Common perineal (fibular), fibular neck
21
Q
What nerve and muscle can result in trendelenurg sign/gait? Which side is injured relative to the sign
A
Superior gluteal nerve to the gluteus medius, lesion is on the opposite side that the lower hip is on
22
Q
Which nerve everts the foot? Which inverts?
A
Peroneal everts, tibial inverts
23
Q
What is a good landmark for pudendal nerve anesthesia?
A
Ischial spine
24
Q
What artery is associated with the axillary nerve at the surgical neck of the humerus?
A
Posterior circumflex artery
25
What artery is associated with the long thoracic nerve?
Lateral thoraci artery
26
Where would you find the tibial nerve and artery at the ankle?
Posterior to the medial malleolus (tom, dick, and harry)
27
Describe the T-tubules and terminal cistern in skeletal and cardiac muscle
Skeletal: triad- 1 t-tubule+ 2 terminal cisternae
Cardiac: dyad- 1 t-tubule+ 1 terminal cisterna
28
What receptor at the sarcoplasmic reticulum is voltage-sensitive?
Dihydropyridine (it is coupled with the ryanodine receptor)
29
What causes the muscle power stroke?
Release of bound ADP and Pi by myosin
30
Which bands shorten in muscle contraction?
H band and I band (also distance between Z lines)
31
What amino acid is needed to synthesize arginine?
arginine
32
Is phosphorylated or dephosphorylated smooth muscle myosin actively contracting?
Phosphorylated
33
What type of bone formation is responsible for the base of the skull? Calvarium? Facial bones?
Base of the skull (and long bones): endochondral
| Calvarium and facial bones: membranous ossification
34
Where do osteoblasts come from (cell line)?
Mesenchyme in periosteum
35
Why do achondroplasia patients have a large head relative to limbs? Genetic defect? What happens to homozygotes?
Because endochondral formation is affected and intramembranous ossification is not. Activating mutation of FGFR3. Homozygosity is lethal (AD)
36
What lab values are telling of osteoporosis? What part of the bone is lost?
None, labs are normal. Cortical and trabecular thinning
37
What is denosumab?
Monoclonal antibody against RANK-L
38
What mutation can cause osteopetrosis? What happens to the bone marrow? Tx?
Defective osteoclasts possibly caused by a carbonic anhydrase II mutation. The bone marrow may be filled in causing pancytopenia and extra medullary hematopoiesis. BMT is potentially curative as osteoclasts are derived from monocytes
39
What lab finding will you see in osteomalacia/rickets?
Increased osteoblast activity: increase in ALP (also typically low vitamin D)
40
What stage is first in Paget disease? What are two feared complications?
Osteoclastic. Arteriovenous malformations in new bone may cause high output heart failure. It also increases osteosarcoma risk
41
What artery is responsible for AVN of the femoral head?
Medial circumflex femoral artery
42
Soap-bubble appearance tumor. Where is it? What cell type?
Giant cell tumor. Typically epiphyseal end of long bones. ONLY EPIPHYSEAL TUMOR. Osteoclastoma/ multinucleate giant cells
43
What part of the bone do you expect to see osteosarcoma in? Ewing sarcoma?
Osteosarc: metaphysis
Ewing: diaphysis
44
What tumor is associated with onion skin periosteal reaction in bone? Mutation?
Ewing: t(11:22) (EWS-FLI-1)
45
What HLA is associated with RA? Antibodies? What is RF?
HLA-DR4, anti-CCP and RF( IgM to Fc of IgG)
46
Is osteoarthritis symmetric? Is RA?
RA is, osteoarthritis is not
47
How long must morning stiffness last in RA?
More than 1 hour
48
Does osteoarthritis or RA affect DIP?
Osteoarthritis
49
What would the histology of a rheumatoid nodule show?
Fibrinoid necrosis with palisading histiocytes
50
What is Caplan syndrome?
RA + pneumoconiosis
51
Which glycogen storage disease can cause gout?
Von-Gierke
52
What is the composition of pseudo gout crystals?
Calcium pyrophosphate
53
What finding in the joint, besides crystals is relatively specific for pseudo gout?
Chondrocalcinosis
54
Antibodies with Sjogren syndrome? Late enlarging parotid gland concern?
SS-A (anti-Ro), SS-B (anti-La)
| MALT lymphoma
55
Triad with gonococcal arthritis?
Polyarthralgias, tenosynovitis, dermatitis
56
What are seronegative spondyloarthritis examples (4). What HLA? What does seronegative mean?
PAIR: Psoriatic arthritis, Ankylosing spondylitis, Inflammatory bowel disease, Reiters/Reactive
HLA-B27, no RF
57
Pencil in cup deformity of DIP on x-ray
Psoriatic arthritis
58
Heart problem associated with ankylosing spondylitis?
aortic regurgitation
59
Most common and severe renal involvement with Lupus?
Diffuse proliferative
60
Most common cause of death in lupus?
Cardiovascular disease
61
Which antibodies suggest poor prognosis with SLE?
Anti-dsDNA (renal disease)
62
Antihistone antibodies are sensitive for what?
Drug induced lupus
63
Recurrent thrombosis, spontaneous abortion in autoimmune patient? Necessary lab?
Antiphospholipid syndrome (lupus anticoagulant, anticardiolipin, or anti-beta2 glycoprotein antiboidies)
64
anti-U1 RNP antibodies?
MCTD
65
What finding will be in a sarcoid patient bronchoalveolar lavage fluid sample?
Elevated CD4+/CD8+ ratio
66
Treatment for a 20-50 yr old woman with widespread chronic MSK pain, poor sleep, stiffness, cognitive disturbances.
Fibromyalgia: TCAs, SNRIs, regular exercise, anticonvulsants
67
Antibodies in polymyositis/dermatomyositis?
anti-Jo (anti-histidyl tRNA), anti-SRP, anti-Mi-2
68
What type of cells and muscle inflammation are associated with polymyositis? Dermato?
Dermato: CD 4+ perimysial inflammation
Poly: Endomysial CD8+ inflammation
Both are progressive symmetric proximal muscle weakness
69
Fingertip pitting is associated with what?
Scleroderma
70
What is the most common cause of death with scleroderma?
Pulmonary sclerosis
71
Antibodies with scleroderma and CREST?
Scleroderma: anti-Scl-70 (anti DNA topoisomerase I)
CREST: anti-centromere
72
Color change in raynaud? What is raynaud syndrome vs. disease? Tx?
White to blue to red. Syndrome is secondary to a disease process, disease if primary. Tx with CCBs
73
Which layer of the epidermis has stem cells? What enzyme activity will be high here?
Stratum basale- telomerase
74
What filaments give desmosomes support?
Cytokeratin and desmoplakin
75
What forms adherens junctions? Significance of these junctions?
Cadherins connecting actin cytoskeletons of adjacent cells. Loss of E-cadherins promotes metastasis
76
What is the function of integrins? What do they bind to?
Maintain integrity of basolateral membrane by binding to COLLAGEN (via fibronectin) and LAMININ in the basement membrane
77
What is hyperkeratosis? What is parakeratosis?
Hyperkeratosis is thickening of the stratum corneum while parakaratosis is hyperkeratosis with retention of nuclei in the stratum corneum
78
Where is the edematous fluid in spongiosis?
Epidermis (intercellular spaces)
79
Difference in albinism and vitiligo?
Albinism is a inability to produce melanin (normal melanocyte number), vitiligo is a decrease in melanocyte number due to autoimmune destruction
80
What is another name for atopic dermatitis? What will you see in the serum? Location on body?
Eczema. increased IgE. On face in infants, then in antecubital fossae
81
Acanthosis with parakeratotic scaling and small microabscesses
Psoriasis
82
What layers are thickened in psoriasis? Decreased?
Corneum and spinosum are increased and the granulosum is decreased
83
Inflammatory facial skin with erythema and no comedones. May be associated with facial flushing in response to external stimuli like alcohol or heat.
rosacea
84
What causes hives/urticaria? Where is the edema (fluid)?
Mast cell degranulation, fluid is in the superficial DERMIS
85
What cancer is associated with vinyl chloride and arsenic exposures?
Hepatocellular angiosarcoma
86
How do you distinguish between bacillary angiomatosis and kaposi sarcoma?
BA: neutrophilic infiltrate
Kaposi: lymphocytic infiltrate
87
What should you consider in an ulcerative/bleeding mouth lesion in a pregnant woman?
Pyogenic granuloma (it's benign even though it looks nasty)
88
Which has well defined borders? Erysipelas or cellulitis?
Erysipelas
89
What layers of skin are involved in staphylococcal scalded skin syndrome? TEN/SJS?
SSSS: Destroyed keratinocyte attachments in GRANULOSUM only (Nikolsky sign)
TEN/SJS: dermal-epidermal junction
90
Treatment for dermatitis herpetiformis?
Dapsone, gluten free diet
91
What percent of the body must be involved for it to be classified TEN? SJS?
TEN: >30%
SJS:
92
What type of cancer can actinic keratosis evolve into?
SCC
93
What is inflammatory lesions of subcutaneous fat, usually on anterior shins?
Erythema nodosum
94
Pruritic, purple, polygonal, planar, papules and plaques. What does mucosal involvement look like? Association? Histology?
Lichen Planus, mucosal involvement is Wickham striae
| Sawtooth infiltrate of lymphocytes at dermal-epidermal junction. Associated with hepatitis C
95
"Christmas tree distribution" rash on trunk
Pityriasis rosea (regresses in 6-8 weeks)
96
What type of radiation causes sunburn? Tanning and photo aging?
UVB= sunBurn
| UVA=tAnning and photoAging
97
Pink pearly nodules with telangiectasias,rolled borders and central crusting or ulceration.
Basal cell carcinoma
98
Which skin cancer has palisading nuclei?
BCC
99
Keratin pearls skin cancer? What does it look like grossly?
SCC, ulcerative red lesions with frequent scale
100
What do you suspect in a rapidly growing skin cancer that has a large keratin filled center?
Keratoacanthoma- a SCC variant that grows rapidly and may spontaneously regress over months
101
What mutation is often associated with melanoma? What drug targets this mutation?
```
BRAF V600E (kinase)
vemurafenib is a BRAF kinase inhibitor
```
102
What is a PGI2 drug? PGE1? PGE2? What are PGE2 agonists used for?
```
PGI2= epoprostenol
PGE1= alprostadil
PGE2= dinoprostone, carboprost to increase uterine tone
```
103
What acid base disorder is caused by aspirin toxicity?
Mixed metabolic acidosis-respiratory alkalosis (resp. all early)- note also causes tinnitus
104
What is the effect of NSAIDs on stomach and kidney? How?
Gastric ulcer since PGs protect gastric mucosa and renal ischemia since PGs vasodilator afferent arteriole
105
Leflunomide MOA
Reversibly inhibits dihydroorotate dehydrogenase, preventing pyrimidine synthesis
106
What are bisphosphanates analogs of? What do they do?
Pyrophosphate, bind hydroxyapetite in bone, inhibiting osteoclast activity
107
Odd side effects of bisphosphonates?
Esophagitis, osteonecrosis of the jaw, atypical stress fractures
108
What is teriperatide?
Recombinant PTH
109
What does pegloticase do?
| Probenecid?
Pegloticase: catalyzes uric acid metabolism to allantoin (water-soluble)
Probenecid: prevents reabsorption of uric acid in PCT, also inhibits penicillin secretion (can cause uric acid caliculi)
110
What is infliximab? Adalimumab?
Both are anti-TNF-alpha monoclonal antibodies
111
What is related to most achondroplasia mutations?
Sporadic and related to increased paternal age
112
What causes blue sclerae in OI?
Thinning of scleral collagen reveals underlying choroidal veins
113
What renal problems are associated with osteopetrosis?
Renal tubular acidosis (decreased carbonic anhydrase II results in decreased reabsorption of HCO3-
114
What causes frontal bossing in Rickets?
Osteoid deposition on the skull
115
What can possibly cause the onset of Paget disease of bone?
Viral infection such as paramyxovirus
116
Does Paget disease involve the entire skeleton?
No, it is a localized process
117
Bone pain plus systemic signs of infection
Osteomyelitis
118
What are sequestrum and involucrum?
Sequestrum is bone abscess and involucrum is the sclerosis around it in osteomyelitis
119
What is Gardner syndrome? Where are the tumors?
FAP +osteomas (face) and fibromatosis
120
What are the two main differences in osteoid osteoma and osteoblastoma?
O-O: cortex of long bone and presents as bone pain that RESOLVES WITH ASPIRIN
Osteoblastoma is larger, arises in the vertebrae and presents as bone pain that does NOT RESPOND TO ASPIRIN
121
Most common benign tumor of bone?
Osteochondroma (stalk)
122
Where do osteosarcomas arise?
Metaphysis of long bones
123
What cell line do Ewing Sarcomas arise from?
Neuroectoderm
124
Where do benign cartilage tumors typically originate? Malignant?
Benign: periphery
Malignant: central
125
What metastatic tumor to bone will produce an OSTEOBLASTIC lesion?
Prostate carcinoma
126
What is the major risk factor for osteoarthritis?
age
127
What histology does a rheumatoid nodule resemble?
Caseating granuloma
128
What are two extra-articular manifestations of ankylosing spondylitis?
Uveitis and aortitis (leads to aortic regurgitation)
129
Sausage fingers or toes?
Psoriatic arthritis
130
What might cause dermatomyositis?
It can be a paraneoplastic syndrome (e.g. gastric carcinoma)
131
If you hear lipoblast cell in a soft tissue situation, what should you think?
Liposarcoma
132
What is cardiac rhabdomyoma associated with?
Tuberous sclerosis
133
Cluster of grapes in young girl vagina?
Rhabdomyosarcoma (sarcoma botryoides)
134
What type of HSR is eczematous dermatitis?
Also called atopic dermatitis (type 1 HSR)
135
What type of glands are in acne? Name and classification
Sebaceous glands, holocrine
136
What does propionibacterium acnes do in sebaceous glands?
Produces lipase's that break down sebum, releasing pro inflammatory fatty acids
137
How do benzoyl peroxide and isotretinoin treat acne?
Decrease keratin production
138
What layer of the skin do you see Munro micro abscesses in?
corneum
139
Sawtooth appearance of dermal epidermal junction. Association?
Lichen planus, Chronic hep. C
140
Targetoid rash and bullae, most common association? What if it has oral mucosa/lip involvement and fever?
Erythema multiforme, HSV, SJS
141
What is an odd risk factor for squamous cell carcinoma of the skin?
Immunosuppression (also AK, Arsenic)
142
What causes a freckle (ephelis)?
Increased number of melanosomes (not melanocytes)
143
What is the most common type of mole in adults? Where do nevi begin?
Intradermal, begin at dermal-epidermal junction (junctional)
144
Most important prognostic factor in predicting melanoma metastasis?
Depth of extension
145
What is formed in pseudo glandular phase of lung development? Canalicular? Saccular? When is respiration capable?
Pseudoglandular: terminal bronchioles
Canalicular: respiratory bronchioles and alveolar ducts (respiration possible here at 25 weeks)
Saccular: terminal sacs
146
What is the function of club cells?
Degrade toxins, act as reserve cells.
147
What is the relationship between radius and collapsing pressure? How does surfactant alter the collapsing pressure?
Increased radius means decreased collapsing pressure.
| Surfactant decreases surface tension which decreases collapsing pressure
148
What is the L:S ratio in a lung that is healthy? What is the most important lecithin?
L:S >2 is healthy, the most important in surfactant is dipalmitoylphosphatidylcholine
149
What dos the foam stability index test measure?
Surfactant levels/ fetal lung maturity
150
What is a major complication of neonatal respiratory distress syndrome?
Necrotizing entercolitis
151
What three things can therapeutic O2 supplementation cause in a newborn?
Retinopathy of prematurity, Intraventricular hemorrhage, Bronchopulmonary dysplasia
152
Where does cartilage end? Goblet cells? Smooth muscle? Cilia?
Cartilage and goblet cells to end of bronchi
Smooth muscle to end of terminal bronchioles
Cilia terminate in respiratory bronchioles
153
What is the difference in lung volumes and capacities?
Volumes are non-overlapping, capacities are the sums of multiple lung volumes
154
Normal tidal volume and dead space volume?
Vt=500mL
| Vd=150mL
155
What are alveolar, intrapleural, and airway pressures at FRC?
Alveolar and airway are 0 (no air moving), intrpleural pressure is negative (~-5)
156
What happens to lung compliance in normal aging?
Increases (e.g. COPD)
157
Which form of hemoglobin has high affinity for O2? Low?
Taut has low (deoxygenated), relaxed has high
158
What causes the difference in O2 affinity with fetal hemoglobin?
Decreased affinity for 2,3 BPG (higher affinity for O2 so it can be stolen from mom)
159
Chocolate colored blood. What is it? Can it be useful? Treatment?
Methemoglobinemia (Fe3+ that doesn't bind O2)
Can be used to treat cyanide poisoning (nitrites followed by thiosulfate) because it has a higher affinity for CN than normal hemoglobin
Tx: Methylene blue and Vitamin C
160
What does caroxyhemoglobin do to Hb saturation curve?
Left shift (increased O2 affinity and won't let go)
161
What poisoning can give a burnt almond smell?
Cyanide
162
When might oxygen demonstrate diffusion limited exchange? Will CO2 ever do this?
Fibrosis, emphysema (normally perfusion limited), no CO2 is never diffusion limited
163
What is the normal A-a gradient?
10-15 mmHg
164
Is V/Q ratio normally perfusion or ventilation driven?
Perfusion driven (V/Q is higher at apex and lower at base due to gravitational effects on blood flow)
165
Does 100% O2 help with V/Q of 0? What about infinity?
0=airway obstruction--> O2 doesn't help
| infinity=blood obstruction--> O2 does help
166
How is most CO2 transported in plasma?
As bicarbonate (90%)
167
Is Cl- higher in venous circulation or arterial? Why?
Arterial. At peripheral tissue, HCO3- produced in RBCs from the CO2 that it just picked up. This HCO3- is transported out of the cell in exchange for a chloride ion
168
What kind of acid-base disturbance is seen with moving to high altitude?
Respiratory alkalosis since ventilation increases to try to compensate for the lower oxygen in the air
169
How do PaCO2 and PaO2 change with exercise?
They don't but venous CO2 content and O2 content do change
170
What sinus is typically involved in rhinosinusitis? Where does it drain? Most common cause?
Maxillary sinus that drains into middle meatus. Most common cause is viral URI which may cause superimposed bacterial infection
171
The sphenopalatine artery is a branch of what artery?
Maxillary
172
What is the virchow triad?
Stasis, Hyper-coagulability, Endothelial damage
173
Imaging test of choice for DVT?
Compression ultrasound
174
Do you see lines of Zahn in thrombi formed before or after death? What are the lines?
Before
| pink=fibrin/platelets, red=RBCs
175
What is the classic triad of fat emboli?
Hypoxemia, neurologic abnormalities, petechial rash
176
Does high or low V/Q ratio cause problems in PE?
Low. Although the PE directly causes a high V/Q, it indirectly causes a low V/Q in the areas where blood is shunted. The oxygenation cannot keep up with the blood flow to these areas.
177
What is the Reid index? What is it in chronic bronchitis?
Thickness of mucosal gland layer to thickness of wall between epithelium and cartilage (>50%)
178
Describe centriacinar and panacinar emphysema
Centriacinar: smoking, upper lobes,
Panacinar: alpha-1 antitrypsin deficiency, lower lobes
179
What are Curschmann spirals and Charcot-Leyden Crystals formed from?
Curschmann: shed epithelium
Crystals: breakdown of eosinophils in sputum
180
What test helps diagnose asthma? What cardiac finding accompanies it?
Methacholine challenge, pulsus paridoxicus
181
Scoliosis and obesity can lead to what type of lung disease?
Restrictive
182
What type of HSR is hypersensitivity pneumonitis?
HSR types III and IV
183
Golden-brown fusiform rods resembling dumbbells (iron)
Asbestos bodies (ferruginous bodies)
184
Looks like sarcoid except the person works in aerospace industry and has less systemic symptoms
Berylliosis
185
What pneumoconiosis increases TB susceptibility? How?
Silicosis by disrupting phagolysosomes and impairing macrophages
186
Eggshell calcification of hilar lymph nodes
silicosis
187
What is the first step in ARDS? What cells are responsible for the initial damage?
Endothelial damage due to release of neutrophilic substances toxic to alveolar wall
188
At what pressure can we diagnose pulmonary HTN?
>25 mm Hg at rest for mean pulmonary artery pressure
189
Arteriosclerosis, medial hypertrophy and intimal fibrosis of pulmonary arteries
Pulmonary Hypertension
190
What mutation can cause heritable PAH? What random bug can cause PAH?
inactivating mutation in BMPR2 gene (normally inhibits vascular smooth muscle proliferation). Schistosomiasis
191
When is fremitus over the lungs increased?
Consolidation such as lobar pneumonia
192
Milky appearing pleural effusion is from what?
Thoracic duct injury from trauma or malignancy (contains TGs)
193
Empiric treatment for lung abscess?
Clindamycin
194
Where are aspiration abscesses when: patient is supine, upright?
Supine: posterior right upper lobe or superior right lower lobe
Upright: basal right lower lobe
195
Is cytokeratin positive in mesothelioma? Carcinoma? What about calretinin?
Cytokeratin and calretinin are positive in almost all mesotheliomas and negative in most carcinomas.
196
Is smoking a mesothelioma risk factor?
No
197
Compression of what nerve by a pancoast tumor causes hoarseness?
Recurrent laryngeal
198
Seems like a lot of blood in head and neck, but not lower body?
SVC syndrome due to malignancy or thrombosis from indwelling catheters
199
What cancer is associated with subacute cerebellar degeneration? Mutation?
Small cell, L-myc activation
200
Name 3 activating mutations with adenocarcinoma of the lung. What is an associated physical finding?
KRAS, EGFR, ALK. Hypertrophic osteoarthropathy (clubbing)
201
What lung cancer can secrete B-hCG?
Large cell carcinoma
202
What is guaifenesin?
Expectorant. Things respiratory secretions but does not suppress cough reflex
203
How does N-acetylcysteine work for COPD patients?
Liquifies mucus by disrupting disulfide bonds
204
What drugs are good for aspirin induced asthma?
Antileukotrienes (e.g. montelukast)
205
What is omalizumab?
Binds unbound serum IgE and blocks binding to Fc
206
Inflammation of nasal mucosa with eosinophilic infiltrate.
Allergic rhinitis (associated with asthma and eczema)
207
Nasal polyp in kid? Adult?
Kid: CF
Adult: aspirin intolerant asthma
208
How would you expect an angiofibroma to present?
Adolescent males with profuse epistaxis (benign tumor of nasal mucosa)
209
Benign papillary tumor of the vocal cord?
Laryngeal papilloma (HPV 6,11)
210
Where does a laryngeal carcinoma come from?
Epithelial lining of true vocal cord
211
What form of pneumonia do viruses usually cause?
Atypical (interstitial) pneumonia
212
What is the difference in red and gray hepatization in lobar pneumonia?
Hemorrhage (RBCs) are in red, they are degraded in gray
213
What causes cold hemolytic anemia in mycoplasma? Specifically what Ig and what antigen on RBC?
I antigen (i) on RBC is targeted by IgM
214
Where is primary TB located? Secondary?
Primary in lower lobe, secondary in apex
215
What part of the meninges will TB spread to?
Base of brain
216
What happens to PaCO2 and PaO2 in chronic bronchitis? Why?
PaCO2 increases and PaO2 decreases due to mucus plugs
217
Most common cause of emphysema?
Smoking
218
What might A1AT deficiency cause besides emphysema? Histology?
Liver cirrhosis: mutant (misfolded) A1AT accumulates in the ER of hepatocytes resulting in pink, PAS (+) globules in hepatocytes
219
What are the two alleles for A1AT deficiency?
PiM is normal, PiZ is mutant
PiMZ: usually asymptomatic but up risk with smoking
PiZZ: significant risk for panacinar emphysema and cirrhosis
220
What type of T-cells mediate asthma initiation?
1st exposure causes Th2 cells to secrete IL-4 (class switch to IgE) and IL-5 (attracts eosinophils) and IL-10 (stimulates Th2, inhibits Th1). Reexposure causes IgE-mediated mast cell degranulation
221
What eosinophilic product is a major mediator of asthma inflammation (later stage)
Major basic protein
222
What type of lung problem does allergic bronchopulmonary aspergillosis cause?
Bronchiectasis
223
What induces fibrosis in IPF? What does it initially look like on CT? Eventually?
TGF-beta from injured pneumocytes
| Initially is sub pleural, patchy fibrosis that eventually becomes diffuse with "honeycomb" lung
224
Asteroid/stellate bodies in giant cells?
Sarcoid
225
What if you see salivary and lacrimal gland destruction along with non-caseating granulomas in hilum?
Sarcoid, be careful with Sjogrens
226
Plexiform lesions in lungs
Pulmonary hypertension
227
What is the relation of maternal diabetes to NRDS?
Insulin decreases surfactant production
228
Why do you see necrotizing entercolitis and PDA with NRDS?
NRDS--> hypoxemia--> causes Nec.E and PDA
229
What is the second most frequent cause of lung cancer in US? How are we exposed?
Radon exposure, uranium in the soil decays to radon (ex. can be in basements)
230
Tumor composed of lung tissue and cartilage that may be calcified (in lung)
Bronchial hamartoma
231
What type of lung cancer is not amenable to surgical resection?
Small cell carcinoma
232
What cancer often forms a polyp-like mass in the bronchus?
Carcinoid tumor
233
What is a unique site of metastasis for lung tumors?
Adrenals
234
What serves as the interim kidney for the first semester? What forms the final kidney?
Interim: mesonephros
Final: metanephros
235
What parts of the kidney does the ureteric bud form? What layer is the rest of it from?
From ureter back to collecting ducts. The rest is formed by metanephric mesenchyme (glomerulus through DCT)
236
How does the metanephros part of the kidney form?
Induced by infiltration by the ureteric bud
237
What is a multicystic dysplastic kidney?
Nonfunctional kidney consisting of cysts and connective tissue
238
What is the pattern of blood flow through the kidney?
segmental, intralobar, arcuate, intralobular, afferent arteriole, glomerulus, efferent arteriole,peritubular capillaries/vasa recta
239
What is the JG apparatus?
Modified SM cells between the DCT and afferent arteriole that is responsible for renin secretion
240
What substance can be used to measure plasma volume? Extracellular volume?
Albumin for plasma volume, inulin/mannitol for extracellular volume
241
How much water is in each body compartment?
60% total body water, 40% ICF, 20% ECF
| Plasma=25% ECF
242
Does creatinine clearance over or underestimate GFR? What about PAH with total renal plasma flow?
Creatinine overestimates slightly (some is secreted)
| PAH underestimates slightly (some gets through)
243
Where do prostaglandins and ang. II have their effects on the renal vasculature?
Ang. II constricts the efferent arteriole while PGs dilate the afferent arteriole
244
How is glucose reabsorbed in the PCT?
Na+/glucose cotransport
245
How does PCT reabsorption affect tonicity?
It is isotonic
246
Where does PTH act to secrete phosphate and resorb calcium?
Phosphate in the PCT, Calcium in the DCT (e.g. thiazides)
247
What does angiotensin II do at the PCT?
Stimulates Na+/H+ exchange causing increased Na+, H20 and HCO3- reabsorption
248
How does the TALH cause Mg2+ and Ca2+ reabsorption?
The Na/K/2Cl transporter allows only forward motion of Na and Cl but allows K+ back leak. This causes a positive luminal potential that can drive cations through
249
Is the TALH permeable to water? DCT?
No and no
250
Where does urine become fully dilute?
Early DCT
251
Where is the most ANYTHING reabsorbed? Potentially besides Calcium and magnesium
PCT
252
How do thiazides cause calcium reabsorption?
If the Na-Cl cotransporter is blocked, the only way to provide sodium for the Na/K ATPase is through interstitial exchange of calcium from sodium. This allows more calcium influx from the lumen and the cycle continues (basically PTH does the same thing here)
253
What is fanconi syndrome? What notable drugs can cause it?
Generalized reabsorption defect in the PCT, expired tetracyclines
254
Locations of Bartter, Gitelman, Liddle Syndrome? Inheritance?
```
Alphabetic order
Bartter: TALH (~loop diuretics)
Gitelman: DCT (~thiazides)
Liddle: Collecting ducts (~aldosterone, ACTIVATING)
All are AR except Liddle is AD
```
255
What is syndrome of apparent mineralocorticoid excess? What else can cause it?
Hereditary deficiency of 11beta-hydroxysteroid dehydrogenase which normally converts cortisol to cortisone in aldosterone tissues. If not, cortisol acts on these receptors as if it was aldosterone.
Glycyrrhetinic acid from licorice
256
What happens to inulin concentration in the PCT along the length of it?
It increases in concentration but not amount due to water reabsorption
257
What three stimuli cause increased renin secretion?
```
decreased BP (JG cells)
decreased Na+ delivery to DCT (macula densa)
Beta-1 sympathetic activation (sympathetic tone)
```
258
Why do you not really see reflex bradycardia with angiotensin II?
It limits baroreceptor function
259
Where is Epo released from?
Interstitial cells in peritubular capillary bed
260
What does dopamine do to kidney function?
Secreted by PCT cells and promotes natriuresis.
Low dose: vasodilates everything in kidney
High dose: vasoconstrictor
261
Abdominal pain, kidney stones and mental disturbances?
Hypercalcemia
262
How do you calculate anion gap? What is normal?
Na-(HCO3+Cl), normal is 8-12
263
What should you check for in metabolic alkalosis?
Chloride
264
What is RTA type 1? Type 2? Type 4? Which is hyperkalemia?
Type 1: alpha-intercalated cells don't secrete H+
Type 2: Defect in PCT HCO3- reabsorption
Type 4: Hypoaldosteronism--> hyperkalemia--> decreased NH3 synthesis in PCT and NH4+ excretion
265
In which RTA type is urine pH increased above 5.5?
Type 1, alpha intercalated cells are normally responsible for acidifying the urine
266
What causes type II RTA?
Fanconi syndrome and CAII inhibitors
267
If there are casts in the urine, where must the damage be?
Glomerulus or renal tubules
268
As nephrotic means protein, what does nephritic mean?
Inflammation--> hematuria
269
Starry sky granular appearance on IF? Also called lumpy-bumpy.
PSGN
270
What complement protein is associated with PSGN?
C3, thus serum C3 is decreased
271
Where are the immune complexes deposited in PSGN?
Subepithelial
272
What is the severe renal problem associated with lupus? Characteristic LM finding?
Diffuse proliferative glomerulonephritis.
| Wire looping of capillaries
273
Episodic gross hematuria that occurs concurrently with respiratory or GI tract infections
IgA nephropathy (Berger disease, note NOT BUERGER disease)
274
Alport syndrome inheritance?
X-linked dominant
275
MPGN type I has what appearance on microscopy? What is type I associated with? Type II?
Tram track appearance
Type I: Hepatitis B or C (or idiopathic)
Type II: C3 nephritic factor (stabilizes C3 convertase causing down serum C3 levels)- dense deposit disease
276
What are the clinically significant effects of protein loss in nephrotic syndrome?
1) AT III loss--> hypercoagulability
| 2) Loss of Ig--> increased infection risk
277
What EM appearance do you see with membranous nephropathy (membranous glomerulonephritis)
Spike and dome appearance with subepithelial deposits
278
What antibodies are associated with Membranous Nephropathy?
Antibodies to Phospholipase A2 receptor
279
What is the most common kidney stone? Serum and urine findings?
Calcium oxalate stone in patient with hypercalciuria and normocalemia
280
What two kidney stones precipitate in low pH? High pH?
Low: uric acid and cystine
High: Ammonium magnesium phosphate, calcium phosphate
281
Which kidney stones are radiolucent?
Uric acid and cystine
282
What causes calcium oxalate stones?
Hypocitraturia
283
What do you suspect with a positive sodium cyanide nitroprusside test?
Cystine stones
284
Dilation of renal pelvis and calyces
Hydronephrosis
285
Well circumscribed kidney mass with central scar and large eosinophilic cells. Tx? What cells does it arise from?
Renal oncocytoma, often resected to exclude malignancy
| Arises from collecting duct cells
286
What cell does renal cell carcinoma arise from?
PCT cells
287
What is WAGR complex?
Wilms tumor, Aniridia, GU malformations, Retardation
288
Chromosome for wilms tumor?
11
289
What is suggested by painless hematuria with no casts?
Bladder cancer
290
What can cause squamous cell carcinoma of the bladder?
Chronic irritation--> metaplasia--> cancer
| E.g. schistosoma haematobium
291
Are there systemic signs with urinary tract infections?
No, usually only with pyelonephritis
292
What does positive urine nitrites indicate?
Gram negative bacteria
293
WBC casts and WBCs in urine suggests what?
Acute pyelonephritis
294
Are the glomeruli/vessels or cortex more affected with acute pyelonephritis?
Cortex, glomerulus mostly spared
295
What does thyroidization of the kidney indicate?
Chronic pyelonephritis (usually means something like vesicoureteral reflux or chronically obstructing stones)
296
What is renal osteodystrophy? What area of bones is affected?
Hypocalcemia, hyperphosphatemia, and failure of vitamin D hydroxylation with chronic renal disease. Causes subperiosteal thinning of bones
297
Serum BUN/Cr in prerenal vs. intrinsic renal failure
Prerenal: BUN/Cr >20
Intrinsic: BUN/Cr
298
What type of dyslipidemia is a consequence of renal failure?
Triglyceridemia
299
Pyuria of eosinophils and azotemia?
Acute interstitial nephritis
300
What is the effect on potassium levels of the maintenance and recovery phase of ATN?
Maintenance: risk of hyperkalemia (kidney can't get rid of it)
Recovery: risk of hypokalemia (kidney is working well again)
301
Gross hematuria and proteinuria with sloughing of necrotic renal papillae
Renal papillary necrosis
302
Chromosomes for PKD1 and PKD2 in adult polycyctic kidney disease?
16, 4 respectively
303
What is dilated in AR polycystic kidney disease? Association?
Collecting ducts, hepatic fibrosis and hepatic cysts
304
What diuretic is CI in anuria?
Mannitol
305
Effect of loop diuretics on afferent arteriole?
Stimulate PGE release and thus afferent vasodilation
306
What is ethacrynic acid?
Non-sulfa loop inhibitor. More ototoxic
307
What is metolazone?
Thiazide diuretic
308
What are the effects of thiazides on glucose, lipids, uric acid?
hyper all of them
309
What does inhibition of ACE due to bradykinin?
Prevents inactivation (it is a potent vasodilator)
310
What is aliskiren?
Direct renin inhibitor (blocks angiotensinogen conversion to Ang. I)
311
How is dysplastic kidney inherited?
It isn't, risk in subsequent pregnancy is very low
312
Baby with portal hypertension?
Polycystic kidney disease
313
ARF with oxalate crystals in the urine?
Ethylene glycol toxicity
314
What lipid changes are seen with nephrotic syndrome?
Hyperlipidemia and hypercholesterolemia to keep the osmolarity of the serum high
315
What might MCD be associated with rarely?
Hodgkin lymphoma
316
Which nephrotic syndrome will respond to steroids?
MCD
317
What nephritic and nephrotic syndromes are associated with SLE?
Nephrotic: Membranous nephropathy (caucasian one)
Nephritis: DPGN (severe)
318
Which kidney pathologies are associated with HBV and HCV?
Membranous nephropathy and membranoproliferative glomerulonephritis (type 1: tram tracks)
319
What is C3 nephritic factor? What is it associated with?
It is an autoantibody that stabilizes c3 convertase causing over activation of complement. Associated with dense deposit disease (Type II MPGN)
320
Why do you see microalbuminuria in diabetes?
Preferential hyaline arteriolosclerosis of the efferent arteriole causes hyper filtration (higher filtration pressure)
321
What are crescents composed of in RPGN?
Fibrin and macrophages
322
What type of nephritic syndrome does lupus cause? Broad class and specific?
DPGN which is crescentic glomerulonephritis
323
Can't pee, can't see, can't hear a buzzing bee
Alport syndrome
324
Scarring at upper and lower kidney poles is characteristic of what?
Vesicoureteral reflux
325
What kidney stone is most commonly seen in children?
Cystine stones
326
What occurs with cystic development during dialysis?
Increased risk for renal cell carcinoma
327
What is angiomyolipoma associated with?
Tuberous sclerosis
328
How does VHL mutation cause renal carcinoma?
VHL leads to increased IGF-1 and increased HIF transcription factor which increases VEGF and PDGF
329
Major risk factor for sporadic renal carcinoma?
Cigarette smoke
330
Popular syndrome with Wilms tumor?
WAGR: Wilms, Aniridia, Genital abnormalities and Retardation
331
What are the two pathways to develop urothelial carcinoma? Describe the differences
1) Flat: starts high grade, flat development, EARLY p53 mutation
2) Papillary: low grade to high grade to invasion
332
In what circumstance can an adenocarcinoma arise in the lower urinary tract?
urachal remnant
333
What is the life span of RBCs and platelets?
RBC: 120 days
Platelets: 8-10 days
334
Why are reticulocytes blue on Wright-Giemsa stain?
residual ribosomal RNA
335
Where are platelets stored? How much?
1/3 stored in spleen
336
Where do you find collagenase, lysozyme and lactoferrin? B-glucuronidase?
First three specific granules of neutrophils, last one in azurophilic granules of neutrophils
337
What about macrophages initiates septic shock?
Lipid A from bacterial LPS binds CD14 on macrophages to initiate septic shock
338
What cells contain heparin and histamine?
Basophils and mast cells
339
When do you see basophilia?
CML
340
What MHC can B cells use to function as an APC?
MHC II- important for class switching
341
What is "the Hoff" in plasma cells?
Large golgi apparatus
342
What do HbS and HbC replace glutamate with?
Valine and lysine (+) respectively
343
What is the typical hemophilia inheritance pattern? Exception?
XR, except hemophilia C (factor XI) is AR
344
How does tPA work?
Causes plasminogen to be activated to plasmin which cleaves fibrin clots
345
What enzyme is required to activate vitamin K to be a cofactor for gamma-glutamyl carboxylase?
Epoxide reductase (warfarin targets this VKOR)
346
What clotting factor does vWF carry and protect? Which factor dies first?
VIII, VII
347
What mediates the transient vasoconstriction following endothelial damage?
Endothelin
348
What substance induces GPIIb/IIIa expression on platelets?
ADP
349
What is the MOA of clopidogrel? Ticlopidine? Abciximab? Tirofiban?
First two are ADP receptor blockers and the second two are direct GPIIb/IIIa inhibitors
350
What causes acanthocytes? Echinocytes? Difference?
Acanthocyte: abetalipoproteinemia
Echinocyte: more uniform and smaller projections
Echinocytes have projections that are more uniform and smaller
351
When do you see bite cells?
G6PD deficiency (post heinz body)
352
Where is the excess iron stored in ringed sideroblasts?
Mitochondria
353
What is a heinz body? Howell-Jolly body?
- Hb precipitates
| - nuclear remnants in patients with hyposplenia/asplenia
354
What class of anemia does lead poisoning cause?
Microcytic (copper deficiency does too)
355
Spoon nails are associated with what?
Iron deficiency anemia
356
IDA, esophageal webs, dysphagia?
Plummer-Vinson Syndrome
357
What is HBH, Hb Barts?
HbH is 4 gamma chains and is seem with four allele deletion in alpha thal. Hb Barts is beta tetramers seen in 3 allele deletion in alpha thalassemia?
358
Who is more likely to have a cis deletion of the alpha thalasemia genes?
Asians
359
What is the genetic defect in beta thalassemia?
Point mutations in splice sites and promoter sequences
360
What two enzymes are inhibited by lead poisoning?
ALAD and ferrelchelotase
361
Where do you see lead lines?
Gingivae and metaphyses of long bones
362
What neuro signs do you see with lead poisoning? Treatment (what about kids?)
Foot and wrist drop
| Dimercaprol and EDTA are 1st line, succimer used for chelation in kids
363
Defect in sideroblastic anemia? Causes?
X-linked defect in ALAS. Alcohol, lead, vitamin B6 deficiency, copper deficiency, INH
364
What is the treatment for sideroblastic anemia?
Vitamin B6
365
Defect in orotic aciduria? Blood findings?
Decreased UMP synthase, megaloblastic anemia refractory to folate and cobalamin
366
Difference in Diamond Blackfan anemia and fanconi anemia?
DBA is a defect in erythroid progenitor cells (actual anemia) associated with UE malformations. Fanconi anemia is DNA repair defect causing bone marrow failure, increased tumor chances, thumb/radial defects
367
What causes PNH? Treatment?
Impaired GPI anchor synthesis with holds DAF (CD55/59) onto RBCs. This deactivates complement normally to protect RBCs. Treatment is eculizumab (terminal complement inhibitor)
368
What causes warm hemolytic anemia? Cold?
Warm: IgG--> SLE and CLL
Cold: IgM--> mycoplasma and mono
369
What do corticosteroids do to WBC levels? how?
Increased neutrophils: impaired adhesion and migration out of blood
Decreased esoinophils: sequestered in LN
Decreased lymphocytes: apoptosis
370
Which step of heme synthesis requires vitamin B6 as a cofactor? What inhibits this step?
ALAS, heme/glucose
371
AIP and PCT enzymes?
AIP: PBGD
PCT: UROD
372
How does iron cause cell damage?
Peroxidation of membrane lipids
373
What causes immune thrombocytopenia?
Anti-GPIIb/IIIa antibodies (commonly due to viral illness)
374
What causes TTP? Pentad of sx?
```
ADAMTS13 deficiency (cleaves vWF multimers)
Neuro sx, thrombocytopenia, hemolytic anemia, renal failure, fever
```
375
What is the inheritance of and treatment for vWF deficiency?
AD, treat with desmopressin
376
How do you detect antithrombin deficiency?
No direct effects, but causes a decrease in the PTT change with heparin administration.
377
What factors do proteins C and S target?
Five and eight
378
What electrolyte abnormalities may be caused by blood transfusion?
Hyperkalemia and hypocalcemia (citrate)
379
Which lymphoma type displays: noncontiguous spread? EBV association? Good prognosis?
Non-hodgkin, hodgkin, hodgkin
380
Cell markers for reed-sternberg cells?
CD 15 and 30
381
Cancer with lytic bone lesions and hypercalcemia besides multiple myeloma
Adult T-Cell Lymphoma
382
Atypical CD4+ cells with "cerebriform" nuclei and skin patches/plaques. What can it progress to?
Mycosis fungoides--> Sezary syndrome (T-cell leukemia)
383
Most common non-Hodgkin lymphoma in adults
DLBCL
384
Starry sky appearance? Where is the sporadic form? Mutation?
Burkitt, pelvis or abdomen, t(8:14) c-myc with Ig heavy chain
385
What might an eosinophilic protein in the urine make you consider? Blood finding?
Multiple Myeloma (Bence-Jones), Rouleaux formation
386
Neutrophils with bilobed nuclei: what are they and when do you see them?
Pseudo-Pegler-Huet anomaly and after chemotherapy
387
Indolent, waxing and waning lymphoma. Mutation?
Follicular lymphoma (BCL-2 t(14:18))
388
What does TdT+ mean? CD10+?
Lymphoblast (T or B cells), marker of pre B-cells
389
Which cancers are associated with Down syndrome?
B-ALL, AML
390
What are two odd places that ALL may spread to that require specific treatment? Mutation with good prognosis?
CNS and testes. t(12;21)
391
What do you suspect with CD20+, CD5+ cells? What type of cells are they? Specific finding?
CLL, B-cells, Smudge cells
392
Whats the difference with smudge and hairy cells on appearance?
Smudge cells are odd nuclei, hairy cells are talking about the cytoplasm
393
Hairy cell leukemia: Stains positive for what? Treatment?
TRAP+, Cladribine
394
What is a specific cell finding for AML? What is it? Translation? Treatment? Common presentation
Auer rods (myeloperoxidase), t(15;17), ATRA for treatment. Commonly presents as DIC
395
Basophilia suggests what?
CML
396
How do you differentiate CML neutrophils with a leukemoid reaction?
1) Low LAP as a result of low activity in malignant neutrophils
2) Increased basophils
3) Mutation (t(9;22)= BCR-ABL
397
Lytic bone lesions and skin rash or recurrent otitis media with mass involving mastoid bone. What do cells express?
Langerhans cell histiocytosis, S-100
398
Mutation with myeloproliferative disorders?
V617F JAK2
399
Severe, burning pain and red-blue coloration in extremities?
Erythromyalgia- rare but classic with PV, also possible with ET
400
What cancers can cause inappropriate absolute polycythemia as a paraneoplastic syndrome?
Renal cell carcinoma, hepatocellular carcinoma
401
What are the EPO levels in PV?
Low, feedback suppresses it
402
What does heparin work on?
Antithrombin III which lowers the activity of thrombin and factor Xa
403
Which is used in pregnancy, heparin or warfarin?
Heparin
404
How do you identify LMWH drugs? What do they do?
"-parin" is in name but not heparin. More specific for factor Xa
405
What antibody is associated with HIT?
IgG against platelet factor 4 (PF4)
406
What is bivalirudin?
Direct thrombin inhibitor derived from leech anticoagulant
407
What is streptokinase?
Thrombolytic
408
What is ticlopidine? Side effect?
ADP receptor blocker, neutropenia
409
What are cilostazol and dipyridamole?
Phosphodiesterase III inhibitors causing vasodilation and inhibition of platelet aggregation
410
What are eptifibatide and tirofiban?
GPIIb/IIIa inhibitors ("fib"), also abciximab
411
What activates azathioprine and 6-MP? What do they do?
HGPRT, purine analogs decreasing de novo purine synthesis
412
Side effects of cladribine (3)?
Myelosuppression, nephrotoxicity, neurotoxicity
413
What enzyme does 5-FU inhibit? How does leucovorin affect it?
Thymidylate synthase by covalently complexing folic acid. Leucovorin enhances the activity
414
What does methotrexate do? How does leucovorin affect it?
Dihydrofolate reductase inhibitor that is reversed by leucovorin.
415
What drug specifically acts in the G2 phase?
Bleomycin (free radical formation)
416
What drug is often used in childhood tumors?
Dactinomycin
417
What is the side effect of doxorubicin? What prevents it?
Cardiotoxicity (dilated cardiomyopathy) which can be prevented by dexrazoxane
418
How does cyclophosphamide work? Side effects? What prevents them?
Cross-links DNA at guanine N7.
| Hemorrhagic cystitis prevented by mesna or NAC
419
What drugs are often used for brain tumors?
Nitrosoureas (-ustine, streptozocin)
420
How do you prevent cisplatin toxicity?
Prevent nephrotoxicity with amifostine and chloride diuresis
421
What is the MOA of irinotecan?
Inhibits topoisomerase I
422
What is the MOA of hydroxyurea?
Inhibits ribonucleotide reductase (DNA synthesis)
423
What is bevacizumab?Erlotinib? Cetuximab? Imatinib?
Monoclonal antibody against
| VEGF, EGFR tyrosine kinase inhibitor, EGFR mab, tyrosine kinase inhibitor of BCR-ABL
424
What antibody increases the risk of PML?
Rituximab (CD 20)
425
What is Vemurafenib?
inhibit V600E BRAF mutation (V600E MUtated, bRAF INHIBition)
426
Toxicity of trastuzumab?
Cardiotoxicity ("heartceptin")
427
What does vWF bind to besides platelets? Where does it come from?
Exposed subendothelial collagen. Weibel-Palade bodies and alpha granules of platelets
428
What is ITP caused by? What fixes it?
Plasma cells in the spleen produce IgG against platelet antigens like GPIIb/IIIa. Then spleen removes antibody bound platelets. Treatment is splenectomy
429
What is the effect of uremia on platelets?
Impairs both adhesion and aggregation
430
What activates factors XII and VII?
XII: sub endothelial collagen
VII: Tissue thromboplastin
431
What do you suspect with increased PTT that does not correct upon mixing normal plasma?
Coagulation factor inhibitor
432
Treatment for vWF disease? Inheritance?
AD, desmopressin which increase W-P body vWF release
433
What coagulation time can be watched for liver failure?
PT (factor VII is the first to go)
434
Why might you see thrombosis in HIT?
Because destroyed platelet fragments may activate remaining platelets (sort of paradoxical)
435
Name two odd things that can cause DIC. One is a cancer and the other is a toxin
Adenocarcinoma: mucin activates coagulation
| Rattlesnake bite: venom activates coagulation
436
How does plasmin stop clotting? What stops plasmin?
Plasmin: 1) cleaves fibrin and fibrinogen
2) Destroys coagulation factors
3) Blocks platelet aggregation
It is stopped by alpha 2 antiplasmin
437
Name two things that can cause a disorder of fibrinolysis (plasmin over activation)
1) Radical prostatectomy: urokinase release activates plasmin
2) Cirrhosis: reduced alpha 2 antiplasmin production
438
Increased PT/PTT, increased bleeding time, increased fibrinogen split products. What is the platelet count? What is the treatment?
This is plasmin over activation (no D-dimer, increased FIBRINOGEN split). Normal platelet count. Treat with aminocaproic acid which blocks plasminogen activation
439
How do B12 and folate deficiency increase thrombosis risk?
They increase homocysteine levels by preventing conversion to methionine. Homocysteine damages endothelial cells and promotes thrombosis
440
What enzyme takes homocysteine to cystathionine?
cystathionine beta synthase (CBS)
441
Dyspnea and petichiae on skin?
Fat embolus (neuro sx too)
442
Squamous cells and debris in an embolus
Amniotic fluid embolus
443
What arteries provide dual supply to the lungs?
Pulmonary arteries and bronchial arteries
444
What is the general reason for microcytic anemia?
Decreased hemoglobin production--> extra division of RBC to maintain Hb concentration
445
What is heme composed of?
Iron and protoporphyrin
446
Where is iron absorbed?
Duodenum
447
Most likely causes of IDA in developing world elderly population?
Hookworms: Ancylostoma duodenale and Necator americanus
448
What is increased in erythrocytes in IDA?
Free erythrocyte protoporphyrin (FEP)
449
What causes sideroblastic anemia?
Decreased protoporphyrin synthesis (e.g. lead poisoning)
450
What step in protoporphyrin synthesis requires B6 as a cofactor?
ALAS
451
Where is iron trapped in sideroblastic RBCs? Why?
Iron is in the mitochondria because this is where it would normally join protoporphyrin to make heme
452
What drug can cause sideroblastic anemia? Habit? How?
INH causes B6 deficiency. Alcohol is a mitochondrial poison.
453
What is the state of iron in siderblastic anemia?
Iron overloaded state
454
Are the globin chains made in thalassemia defective?
No, just a decrease in synthesis
455
How many alpha thalassemia genes are there? Chromosome? What about beta?
Four alpha on chromosome 16
| 2 beta on chromosome 11
456
Which thalassemia is due to deletions and which to mutations?
Alpha is primarily due to deletions while beta is due to mutations
457
Which thalassemia is associated with extra medullary hematopoiesis?
Alpha
458
What causes macrocytic anemia generally?
Lack of DNA means RBCs cannot keep dividing and stay larger as a result
459
What is pernicious anemia? What deficiency is caused?
Autoimmune destruction of parietal cells leading to intrinsic factor and thus B12 deficiency
460
What is B12 converting that leads to SCD with deficiency? What step forms the MMA?
methylmalonylic acid to succinyl CoA
| Propionyl CoA to MMA via Propionyl CoA carboxylase
461
What causes hemosiderinuria?
Renal tubular cells pick up some of the hemoglobin that is filtered into urine after intravascular hemolysis and break it down into iron. After cell builds up enough iron as hemosiderin it is shed and results in hemosiderinuria
462
Most common cause of death in adult sickle cell patients
Acute Chest Syndrome (vaso-occlusion in pulmonary microcirculation)
463
Where does sickling still occur in sickle cell trait?
Renal medulla (extreme hypoxia and hypertonicity)
464
What does the metabisulfate screen do?
Any cells with any amount of HbS sickle (trait and disease)
465
Why does PNH happen at night?
Mild respiratory acidosis with shallow breathing at night activates complement
466
What is the main cause of death in PNH? What cancer can develop and why?
Thrombosis of hepatic, portal or cerebral veins due to destroyed platelets releasing cytoplasmic contents
- AML, PNH is a defect in myeloid stem cells just like AML
467
Hemoglobinuria and back pain several hours after getting dapsone tx?
G6PD deficiency. Back pain since Hb is nephrotoxic
468
What is the cell mark on a heatopoietic stem cell that leads to myeloid and lymphoid cell lines?
CD34+
469
Why would severe infection cause leukopenia?
Increased movement of neutrophils into tissues
470
What is the most sensitive cell in the body to radiation?
Lymphocytes
471
What cell line leads to erythroblasts?
Myeloid stem cell
472
Decrease in what receptor indicates immature leukocytes?
Fc receptors (CD 16)
473
What is a notable case where you see eosinophilia? Basophilia?
Eosinophilia in Hodgkins lymphoma, basophilia in CML
474
What bacteria can produce lymphocytic leukocytosis?
Bordatella pertussis
475
What causes LAD in mononucleosis? Splenomegaly?
T-cell (CD8+) hyperplasia in lymph node paracortex and Periarterial lymphatic sheath (PALS)
476
What is TdT? When do you see it?
Lymphoblasts and it is a DNA polymerase
477
Which tumor is with down syndrome before 5 years old? After?
Before is AM (megakaryoblastic)L, After is ALL (LL= later later)
478
Leukemia that classically infiltrates gums?
Acute monocytic leukemia
479
What type of leukemia might myelodysplastic syndromes develop into?
AML
480
What do CLL cells express in regards to cell markers? What type of cells are they?
B cells that coexpress CD5 (normally on T cells) and CD20
481
Why do you get splenomegaly in Hairy Cell Leukemia?
They accumulate in the RED pulp (this is odd because WBCs normally live in white pulp)
482
What if you see lytic lesions, HSM, hypercalcemia and a rash?
ATLL (HTLV associated)
483
Aggregates of neoplastic CD4+ cells in the epidermis?
Pautrier micro abscesses with mycosis fungiodes
484
What can CML progress to? Why?
AML or ALL because the mutation is in a pluripotent stem cell
485
Does ET progress to marrow fibrosis or leukemia? Cause gout?
No and no, unlike other MPDs
486
What proliferates in myelofibrosis?
Megakaryocytets which produce excess PDGF and cause fibrosis of the marrow
487
In a lymph node draining a tissue with cancer, what area is hyperplastic?
Medulla (sinus histiocytes)
488
What three diseases are associated with marginal zone lymphoma? Another name of it? When is the marginal zone present?
Only present in chronic inflammation
| H. pylori (MALToma), Hashimoto, Sjogren
489
Most common HL subtype? Best prognosis? Eosinophils?
Nodular sclerosis- most common
Lymphocyte-rich- best prognosis
Mixed cellularity- eosinophils
490
What IL might be high with multiple myeloma?
IL-6 stimulates plasma cell growth and Ig production
491
How does multiple myeloma cause bone lesions?
Activates RANK receptor on osteoclasts
492
What markers are positive in LCH?
CD1a+ and S100+
