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Step1 > Step Review 2: Musculo, Respiratory, Renal, Hem Onc > Flashcards

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

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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

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3
Q

What bursa gets inflamed from excessive kneeling?

A

Prepatellar bursa (e.g. tile layer)

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4
Q

What bursa does a Baker cyst involve?

A

Gastrocnemius/semimembranosus bursa

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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

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6
Q

Which is closer to the thumb: scaphoid or trapezium?

A

Trapezium

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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)

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8
Q

Dislocation of which wrist bone may cause carpal tunnel syndrome? What might cause bilateral carpal tunnel?

A

Lunate, dialysis-related amyloidosis

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9
Q

Is sensation compromised in carpal tunnel?

A

No, the palmar cutaneous branch enters the hand external to the carpal tunnel.

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10
Q

Which ligament is cut to relieve carpal tunnel syndrome?

A

Transverse carpal ligament

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11
Q

What nerve does sensation over the lateral arm? Lateral forearm?

A

Lateral arm= axillary

Lateral forearm= musculocutaneous

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12
Q

What specific branch of the median nerve supplies the thumb?

A

The recurrent branch

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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)

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14
Q

On the posterior hand, does the radial or median nerve do the tips of the thumb side fingers?

A

Median

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15
Q

Which nerve causes a winged scapula?

A

Long thoracic nerve (C5-C7)

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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

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17
Q

What is the action of lumbricals?

A

Extend DIP, PIP and flex MCP

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18
Q

What is compressed in thoracic outlet syndrome? Where?

A

Lower trunk of brachial plexus and subclavian vessels (scalene triangle)

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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

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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

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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

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22
Q

Which nerve everts the foot? Which inverts?

A

Peroneal everts, tibial inverts

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23
Q

What is a good landmark for pudendal nerve anesthesia?

A

Ischial spine

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24
Q

What artery is associated with the axillary nerve at the surgical neck of the humerus?

A

Posterior circumflex artery

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25
Q

What artery is associated with the long thoracic nerve?

A

Lateral thoraci artery

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26
Q

Where would you find the tibial nerve and artery at the ankle?

A

Posterior to the medial malleolus (tom, dick, and harry)

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27
Q

Describe the T-tubules and terminal cistern in skeletal and cardiac muscle

A

Skeletal: triad- 1 t-tubule+ 2 terminal cisternae
Cardiac: dyad- 1 t-tubule+ 1 terminal cisterna

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28
Q

What receptor at the sarcoplasmic reticulum is voltage-sensitive?

A

Dihydropyridine (it is coupled with the ryanodine receptor)

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29
Q

What causes the muscle power stroke?

A

Release of bound ADP and Pi by myosin

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30
Q

Which bands shorten in muscle contraction?

A

H band and I band (also distance between Z lines)

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31
Q

What amino acid is needed to synthesize arginine?

A

arginine

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32
Q

Is phosphorylated or dephosphorylated smooth muscle myosin actively contracting?

A

Phosphorylated

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33
Q

What type of bone formation is responsible for the base of the skull? Calvarium? Facial bones?

A

Base of the skull (and long bones): endochondral

Calvarium and facial bones: membranous ossification

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34
Q

Where do osteoblasts come from (cell line)?

A

Mesenchyme in periosteum

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35
Q

Why do achondroplasia patients have a large head relative to limbs? Genetic defect? What happens to homozygotes?

A

Because endochondral formation is affected and intramembranous ossification is not. Activating mutation of FGFR3. Homozygosity is lethal (AD)

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36
Q

What lab values are telling of osteoporosis? What part of the bone is lost?

A

None, labs are normal. Cortical and trabecular thinning

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37
Q

What is denosumab?

A

Monoclonal antibody against RANK-L

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38
Q

What mutation can cause osteopetrosis? What happens to the bone marrow? Tx?

A

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

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39
Q

What lab finding will you see in osteomalacia/rickets?

A

Increased osteoblast activity: increase in ALP (also typically low vitamin D)

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40
Q

What stage is first in Paget disease? What are two feared complications?

A

Osteoclastic. Arteriovenous malformations in new bone may cause high output heart failure. It also increases osteosarcoma risk

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41
Q

What artery is responsible for AVN of the femoral head?

A

Medial circumflex femoral artery

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42
Q

Soap-bubble appearance tumor. Where is it? What cell type?

A

Giant cell tumor. Typically epiphyseal end of long bones. ONLY EPIPHYSEAL TUMOR. Osteoclastoma/ multinucleate giant cells

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43
Q

What part of the bone do you expect to see osteosarcoma in? Ewing sarcoma?

A

Osteosarc: metaphysis
Ewing: diaphysis

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44
Q

What tumor is associated with onion skin periosteal reaction in bone? Mutation?

A

Ewing: t(11:22) (EWS-FLI-1)

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45
Q

What HLA is associated with RA? Antibodies? What is RF?

A

HLA-DR4, anti-CCP and RF( IgM to Fc of IgG)

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46
Q

Is osteoarthritis symmetric? Is RA?

A

RA is, osteoarthritis is not

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47
Q

How long must morning stiffness last in RA?

A

More than 1 hour

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48
Q

Does osteoarthritis or RA affect DIP?

A

Osteoarthritis

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49
Q

What would the histology of a rheumatoid nodule show?

A

Fibrinoid necrosis with palisading histiocytes

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50
Q

What is Caplan syndrome?

A

RA + pneumoconiosis

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51
Q

Which glycogen storage disease can cause gout?

A

Von-Gierke

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52
Q

What is the composition of pseudo gout crystals?

A

Calcium pyrophosphate

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53
Q

What finding in the joint, besides crystals is relatively specific for pseudo gout?

A

Chondrocalcinosis

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54
Q

Antibodies with Sjogren syndrome? Late enlarging parotid gland concern?

A

SS-A (anti-Ro), SS-B (anti-La)

MALT lymphoma

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55
Q

Triad with gonococcal arthritis?

A

Polyarthralgias, tenosynovitis, dermatitis

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56
Q

What are seronegative spondyloarthritis examples (4). What HLA? What does seronegative mean?

A

PAIR: Psoriatic arthritis, Ankylosing spondylitis, Inflammatory bowel disease, Reiters/Reactive
HLA-B27, no RF

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57
Q

Pencil in cup deformity of DIP on x-ray

A

Psoriatic arthritis

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58
Q

Heart problem associated with ankylosing spondylitis?

A

aortic regurgitation

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59
Q

Most common and severe renal involvement with Lupus?

A

Diffuse proliferative

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60
Q

Most common cause of death in lupus?

A

Cardiovascular disease

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61
Q

Which antibodies suggest poor prognosis with SLE?

A

Anti-dsDNA (renal disease)

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62
Q

Antihistone antibodies are sensitive for what?

A

Drug induced lupus

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63
Q

Recurrent thrombosis, spontaneous abortion in autoimmune patient? Necessary lab?

A

Antiphospholipid syndrome (lupus anticoagulant, anticardiolipin, or anti-beta2 glycoprotein antiboidies)

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64
Q

anti-U1 RNP antibodies?

A

MCTD

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65
Q

What finding will be in a sarcoid patient bronchoalveolar lavage fluid sample?

A

Elevated CD4+/CD8+ ratio

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66
Q

Treatment for a 20-50 yr old woman with widespread chronic MSK pain, poor sleep, stiffness, cognitive disturbances.

A

Fibromyalgia: TCAs, SNRIs, regular exercise, anticonvulsants

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67
Q

Antibodies in polymyositis/dermatomyositis?

A

anti-Jo (anti-histidyl tRNA), anti-SRP, anti-Mi-2

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68
Q

What type of cells and muscle inflammation are associated with polymyositis? Dermato?

A

Dermato: CD 4+ perimysial inflammation
Poly: Endomysial CD8+ inflammation
Both are progressive symmetric proximal muscle weakness

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69
Q

Fingertip pitting is associated with what?

A

Scleroderma

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70
Q

What is the most common cause of death with scleroderma?

A

Pulmonary sclerosis

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71
Q

Antibodies with scleroderma and CREST?

A

Scleroderma: anti-Scl-70 (anti DNA topoisomerase I)
CREST: anti-centromere

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72
Q

Color change in raynaud? What is raynaud syndrome vs. disease? Tx?

A

White to blue to red. Syndrome is secondary to a disease process, disease if primary. Tx with CCBs

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73
Q

Which layer of the epidermis has stem cells? What enzyme activity will be high here?

A

Stratum basale- telomerase

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74
Q

What filaments give desmosomes support?

A

Cytokeratin and desmoplakin

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75
Q

What forms adherens junctions? Significance of these junctions?

A

Cadherins connecting actin cytoskeletons of adjacent cells. Loss of E-cadherins promotes metastasis

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76
Q

What is the function of integrins? What do they bind to?

A

Maintain integrity of basolateral membrane by binding to COLLAGEN (via fibronectin) and LAMININ in the basement membrane

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77
Q

What is hyperkeratosis? What is parakeratosis?

A

Hyperkeratosis is thickening of the stratum corneum while parakaratosis is hyperkeratosis with retention of nuclei in the stratum corneum

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78
Q

Where is the edematous fluid in spongiosis?

A

Epidermis (intercellular spaces)

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79
Q

Difference in albinism and vitiligo?

A

Albinism is a inability to produce melanin (normal melanocyte number), vitiligo is a decrease in melanocyte number due to autoimmune destruction

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80
Q

What is another name for atopic dermatitis? What will you see in the serum? Location on body?

A

Eczema. increased IgE. On face in infants, then in antecubital fossae

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81
Q

Acanthosis with parakeratotic scaling and small microabscesses

A

Psoriasis

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82
Q

What layers are thickened in psoriasis? Decreased?

A

Corneum and spinosum are increased and the granulosum is decreased

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83
Q

Inflammatory facial skin with erythema and no comedones. May be associated with facial flushing in response to external stimuli like alcohol or heat.

A

rosacea

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84
Q

What causes hives/urticaria? Where is the edema (fluid)?

A

Mast cell degranulation, fluid is in the superficial DERMIS

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85
Q

What cancer is associated with vinyl chloride and arsenic exposures?

A

Hepatocellular angiosarcoma

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86
Q

How do you distinguish between bacillary angiomatosis and kaposi sarcoma?

A

BA: neutrophilic infiltrate
Kaposi: lymphocytic infiltrate

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87
Q

What should you consider in an ulcerative/bleeding mouth lesion in a pregnant woman?

A

Pyogenic granuloma (it’s benign even though it looks nasty)

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88
Q

Which has well defined borders? Erysipelas or cellulitis?

A

Erysipelas

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89
Q

What layers of skin are involved in staphylococcal scalded skin syndrome? TEN/SJS?

A

SSSS: Destroyed keratinocyte attachments in GRANULOSUM only (Nikolsky sign)
TEN/SJS: dermal-epidermal junction

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90
Q

Treatment for dermatitis herpetiformis?

A

Dapsone, gluten free diet

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91
Q

What percent of the body must be involved for it to be classified TEN? SJS?

A

TEN: >30%
SJS:

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92
Q

What type of cancer can actinic keratosis evolve into?

A

SCC

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93
Q

What is inflammatory lesions of subcutaneous fat, usually on anterior shins?

A

Erythema nodosum

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94
Q

Pruritic, purple, polygonal, planar, papules and plaques. What does mucosal involvement look like? Association? Histology?

A

Lichen Planus, mucosal involvement is Wickham striae

Sawtooth infiltrate of lymphocytes at dermal-epidermal junction. Associated with hepatitis C

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95
Q

“Christmas tree distribution” rash on trunk

A

Pityriasis rosea (regresses in 6-8 weeks)

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96
Q

What type of radiation causes sunburn? Tanning and photo aging?

A

UVB= sunBurn

UVA=tAnning and photoAging

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97
Q

Pink pearly nodules with telangiectasias,rolled borders and central crusting or ulceration.

A

Basal cell carcinoma

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98
Q

Which skin cancer has palisading nuclei?

A

BCC

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99
Q

Keratin pearls skin cancer? What does it look like grossly?

A

SCC, ulcerative red lesions with frequent scale

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100
Q

What do you suspect in a rapidly growing skin cancer that has a large keratin filled center?

A

Keratoacanthoma- a SCC variant that grows rapidly and may spontaneously regress over months

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101
Q

What mutation is often associated with melanoma? What drug targets this mutation?

A 
BRAF V600E (kinase)
vemurafenib is a BRAF kinase inhibitor
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102
Q

What is a PGI2 drug? PGE1? PGE2? What are PGE2 agonists used for?

A 
PGI2= epoprostenol
PGE1= alprostadil
PGE2= dinoprostone, carboprost to increase uterine tone
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103
Q

What acid base disorder is caused by aspirin toxicity?

A

Mixed metabolic acidosis-respiratory alkalosis (resp. all early)- note also causes tinnitus

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104
Q

What is the effect of NSAIDs on stomach and kidney? How?

A

Gastric ulcer since PGs protect gastric mucosa and renal ischemia since PGs vasodilator afferent arteriole

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105
Q

Leflunomide MOA

A

Reversibly inhibits dihydroorotate dehydrogenase, preventing pyrimidine synthesis

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106
Q

What are bisphosphanates analogs of? What do they do?

A

Pyrophosphate, bind hydroxyapetite in bone, inhibiting osteoclast activity

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107
Q

Odd side effects of bisphosphonates?

A

Esophagitis, osteonecrosis of the jaw, atypical stress fractures

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108
Q

What is teriperatide?

A

Recombinant PTH

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109
Q

What does pegloticase do?

Probenecid?

A

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)

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110
Q

What is infliximab? Adalimumab?

A

Both are anti-TNF-alpha monoclonal antibodies

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111
Q

What is related to most achondroplasia mutations?

A

Sporadic and related to increased paternal age

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112
Q

What causes blue sclerae in OI?

A

Thinning of scleral collagen reveals underlying choroidal veins

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113
Q

What renal problems are associated with osteopetrosis?

A

Renal tubular acidosis (decreased carbonic anhydrase II results in decreased reabsorption of HCO3-

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114
Q

What causes frontal bossing in Rickets?

A

Osteoid deposition on the skull

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115
Q

What can possibly cause the onset of Paget disease of bone?

A

Viral infection such as paramyxovirus

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116
Q

Does Paget disease involve the entire skeleton?

A

No, it is a localized process

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117
Q

Bone pain plus systemic signs of infection

A

Osteomyelitis

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118
Q

What are sequestrum and involucrum?

A

Sequestrum is bone abscess and involucrum is the sclerosis around it in osteomyelitis

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119
Q

What is Gardner syndrome? Where are the tumors?

A

FAP +osteomas (face) and fibromatosis

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120
Q

What are the two main differences in osteoid osteoma and osteoblastoma?

A

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

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121
Q

Most common benign tumor of bone?

A

Osteochondroma (stalk)

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122
Q

Where do osteosarcomas arise?

A

Metaphysis of long bones

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123
Q

What cell line do Ewing Sarcomas arise from?

A

Neuroectoderm

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124
Q

Where do benign cartilage tumors typically originate? Malignant?

A

Benign: periphery
Malignant: central

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125
Q

What metastatic tumor to bone will produce an OSTEOBLASTIC lesion?

A

Prostate carcinoma

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126
Q

What is the major risk factor for osteoarthritis?

A

age

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127
Q

What histology does a rheumatoid nodule resemble?

A

Caseating granuloma

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128
Q

What are two extra-articular manifestations of ankylosing spondylitis?

A

Uveitis and aortitis (leads to aortic regurgitation)

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129
Q

Sausage fingers or toes?

A

Psoriatic arthritis

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130
Q

What might cause dermatomyositis?

A

It can be a paraneoplastic syndrome (e.g. gastric carcinoma)

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131
Q

If you hear lipoblast cell in a soft tissue situation, what should you think?

A

Liposarcoma

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132
Q

What is cardiac rhabdomyoma associated with?

A

Tuberous sclerosis

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133
Q

Cluster of grapes in young girl vagina?

A

Rhabdomyosarcoma (sarcoma botryoides)

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134
Q

What type of HSR is eczematous dermatitis?

A

Also called atopic dermatitis (type 1 HSR)

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135
Q

What type of glands are in acne? Name and classification

A

Sebaceous glands, holocrine

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136
Q

What does propionibacterium acnes do in sebaceous glands?

A

Produces lipase’s that break down sebum, releasing pro inflammatory fatty acids

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137
Q

How do benzoyl peroxide and isotretinoin treat acne?

A

Decrease keratin production

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138
Q

What layer of the skin do you see Munro micro abscesses in?

A

corneum

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139
Q

Sawtooth appearance of dermal epidermal junction. Association?

A

Lichen planus, Chronic hep. C

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140
Q

Targetoid rash and bullae, most common association? What if it has oral mucosa/lip involvement and fever?

A

Erythema multiforme, HSV, SJS

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141
Q

What is an odd risk factor for squamous cell carcinoma of the skin?

A

Immunosuppression (also AK, Arsenic)

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142
Q

What causes a freckle (ephelis)?

A

Increased number of melanosomes (not melanocytes)

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143
Q

What is the most common type of mole in adults? Where do nevi begin?

A

Intradermal, begin at dermal-epidermal junction (junctional)

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144
Q

Most important prognostic factor in predicting melanoma metastasis?

A

Depth of extension

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145
Q

What is formed in pseudo glandular phase of lung development? Canalicular? Saccular? When is respiration capable?

A

Pseudoglandular: terminal bronchioles
Canalicular: respiratory bronchioles and alveolar ducts (respiration possible here at 25 weeks)
Saccular: terminal sacs

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146
Q

What is the function of club cells?

A

Degrade toxins, act as reserve cells.

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147
Q

What is the relationship between radius and collapsing pressure? How does surfactant alter the collapsing pressure?

A

Increased radius means decreased collapsing pressure.

Surfactant decreases surface tension which decreases collapsing pressure

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148
Q

What is the L:S ratio in a lung that is healthy? What is the most important lecithin?

A

L:S >2 is healthy, the most important in surfactant is dipalmitoylphosphatidylcholine

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149
Q

What dos the foam stability index test measure?

A

Surfactant levels/ fetal lung maturity

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150
Q

What is a major complication of neonatal respiratory distress syndrome?

A

Necrotizing entercolitis

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151
Q

What three things can therapeutic O2 supplementation cause in a newborn?

A

Retinopathy of prematurity, Intraventricular hemorrhage, Bronchopulmonary dysplasia

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152
Q

Where does cartilage end? Goblet cells? Smooth muscle? Cilia?

A

Cartilage and goblet cells to end of bronchi
Smooth muscle to end of terminal bronchioles
Cilia terminate in respiratory bronchioles

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153
Q

What is the difference in lung volumes and capacities?

A

Volumes are non-overlapping, capacities are the sums of multiple lung volumes

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154
Q

Normal tidal volume and dead space volume?

A

Vt=500mL

Vd=150mL

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155
Q

What are alveolar, intrapleural, and airway pressures at FRC?

A

Alveolar and airway are 0 (no air moving), intrpleural pressure is negative (~-5)

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156
Q

What happens to lung compliance in normal aging?

A

Increases (e.g. COPD)

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157
Q

Which form of hemoglobin has high affinity for O2? Low?

A

Taut has low (deoxygenated), relaxed has high

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158
Q

What causes the difference in O2 affinity with fetal hemoglobin?

A

Decreased affinity for 2,3 BPG (higher affinity for O2 so it can be stolen from mom)

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159
Q

Chocolate colored blood. What is it? Can it be useful? Treatment?

A

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

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160
Q

What does caroxyhemoglobin do to Hb saturation curve?

A

Left shift (increased O2 affinity and won’t let go)

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161
Q

What poisoning can give a burnt almond smell?

A

Cyanide

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162
Q

When might oxygen demonstrate diffusion limited exchange? Will CO2 ever do this?

A

Fibrosis, emphysema (normally perfusion limited), no CO2 is never diffusion limited

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163
Q

What is the normal A-a gradient?

A

10-15 mmHg

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164
Q

Is V/Q ratio normally perfusion or ventilation driven?

A

Perfusion driven (V/Q is higher at apex and lower at base due to gravitational effects on blood flow)

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165
Q

Does 100% O2 help with V/Q of 0? What about infinity?

A

0=airway obstruction–> O2 doesn’t help

infinity=blood obstruction–> O2 does help

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166
Q

How is most CO2 transported in plasma?

A

As bicarbonate (90%)

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167
Q

Is Cl- higher in venous circulation or arterial? Why?

A

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

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168
Q

What kind of acid-base disturbance is seen with moving to high altitude?

A

Respiratory alkalosis since ventilation increases to try to compensate for the lower oxygen in the air

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169
Q

How do PaCO2 and PaO2 change with exercise?

A

They don’t but venous CO2 content and O2 content do change

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170
Q

What sinus is typically involved in rhinosinusitis? Where does it drain? Most common cause?

A

Maxillary sinus that drains into middle meatus. Most common cause is viral URI which may cause superimposed bacterial infection

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171
Q

The sphenopalatine artery is a branch of what artery?

A

Maxillary

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172
Q

What is the virchow triad?

A

Stasis, Hyper-coagulability, Endothelial damage

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173
Q

Imaging test of choice for DVT?

A

Compression ultrasound

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174
Q

Do you see lines of Zahn in thrombi formed before or after death? What are the lines?

A

Before

pink=fibrin/platelets, red=RBCs

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175
Q

What is the classic triad of fat emboli?

A

Hypoxemia, neurologic abnormalities, petechial rash

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176
Q

Does high or low V/Q ratio cause problems in PE?

A

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.

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177
Q

What is the Reid index? What is it in chronic bronchitis?

A

Thickness of mucosal gland layer to thickness of wall between epithelium and cartilage (>50%)

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178
Q

Describe centriacinar and panacinar emphysema

A

Centriacinar: smoking, upper lobes,
Panacinar: alpha-1 antitrypsin deficiency, lower lobes

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179
Q

What are Curschmann spirals and Charcot-Leyden Crystals formed from?

A

Curschmann: shed epithelium
Crystals: breakdown of eosinophils in sputum

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180
Q

What test helps diagnose asthma? What cardiac finding accompanies it?

A

Methacholine challenge, pulsus paridoxicus

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181
Q

Scoliosis and obesity can lead to what type of lung disease?

A

Restrictive

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182
Q

What type of HSR is hypersensitivity pneumonitis?

A

HSR types III and IV

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183
Q

Golden-brown fusiform rods resembling dumbbells (iron)

A

Asbestos bodies (ferruginous bodies)

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184
Q

Looks like sarcoid except the person works in aerospace industry and has less systemic symptoms

A

Berylliosis

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185
Q

What pneumoconiosis increases TB susceptibility? How?

A

Silicosis by disrupting phagolysosomes and impairing macrophages

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186
Q

Eggshell calcification of hilar lymph nodes

A

silicosis

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187
Q

What is the first step in ARDS? What cells are responsible for the initial damage?

A

Endothelial damage due to release of neutrophilic substances toxic to alveolar wall

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188
Q

At what pressure can we diagnose pulmonary HTN?

A

> 25 mm Hg at rest for mean pulmonary artery pressure

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189
Q

Arteriosclerosis, medial hypertrophy and intimal fibrosis of pulmonary arteries

A

Pulmonary Hypertension

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190
Q

What mutation can cause heritable PAH? What random bug can cause PAH?

A

inactivating mutation in BMPR2 gene (normally inhibits vascular smooth muscle proliferation). Schistosomiasis

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191
Q

When is fremitus over the lungs increased?

A

Consolidation such as lobar pneumonia

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192
Q

Milky appearing pleural effusion is from what?

A

Thoracic duct injury from trauma or malignancy (contains TGs)

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193
Q

Empiric treatment for lung abscess?

A

Clindamycin

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194
Q

Where are aspiration abscesses when: patient is supine, upright?

A

Supine: posterior right upper lobe or superior right lower lobe
Upright: basal right lower lobe

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195
Q

Is cytokeratin positive in mesothelioma? Carcinoma? What about calretinin?

A

Cytokeratin and calretinin are positive in almost all mesotheliomas and negative in most carcinomas.

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196
Q

Is smoking a mesothelioma risk factor?

A

No

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197
Q

Compression of what nerve by a pancoast tumor causes hoarseness?

A

Recurrent laryngeal

198
Q

Seems like a lot of blood in head and neck, but not lower body?

A

SVC syndrome due to malignancy or thrombosis from indwelling catheters

199
Q

What cancer is associated with subacute cerebellar degeneration? Mutation?

A

Small cell, L-myc activation

200
Q

Name 3 activating mutations with adenocarcinoma of the lung. What is an associated physical finding?

A

KRAS, EGFR, ALK. Hypertrophic osteoarthropathy (clubbing)

201
Q

What lung cancer can secrete B-hCG?

A

Large cell carcinoma

202
Q

What is guaifenesin?

A

Expectorant. Things respiratory secretions but does not suppress cough reflex

203
Q

How does N-acetylcysteine work for COPD patients?

A

Liquifies mucus by disrupting disulfide bonds

204
Q

What drugs are good for aspirin induced asthma?

A

Antileukotrienes (e.g. montelukast)

205
Q

What is omalizumab?

A

Binds unbound serum IgE and blocks binding to Fc

206
Q

Inflammation of nasal mucosa with eosinophilic infiltrate.

A

Allergic rhinitis (associated with asthma and eczema)

207
Q

Nasal polyp in kid? Adult?

A

Kid: CF
Adult: aspirin intolerant asthma

208
Q

How would you expect an angiofibroma to present?

A

Adolescent males with profuse epistaxis (benign tumor of nasal mucosa)

209
Q

Benign papillary tumor of the vocal cord?

A

Laryngeal papilloma (HPV 6,11)

210
Q

Where does a laryngeal carcinoma come from?

A

Epithelial lining of true vocal cord

211
Q

What form of pneumonia do viruses usually cause?

A

Atypical (interstitial) pneumonia

212
Q

What is the difference in red and gray hepatization in lobar pneumonia?

A

Hemorrhage (RBCs) are in red, they are degraded in gray

213
Q

What causes cold hemolytic anemia in mycoplasma? Specifically what Ig and what antigen on RBC?

A

I antigen (i) on RBC is targeted by IgM

214
Q

Where is primary TB located? Secondary?

A

Primary in lower lobe, secondary in apex

215
Q

What part of the meninges will TB spread to?

A

Base of brain

216
Q

What happens to PaCO2 and PaO2 in chronic bronchitis? Why?

A

PaCO2 increases and PaO2 decreases due to mucus plugs

217
Q

Most common cause of emphysema?

A

Smoking

218
Q

What might A1AT deficiency cause besides emphysema? Histology?

A

Liver cirrhosis: mutant (misfolded) A1AT accumulates in the ER of hepatocytes resulting in pink, PAS (+) globules in hepatocytes

219
Q

What are the two alleles for A1AT deficiency?

A

PiM is normal, PiZ is mutant
PiMZ: usually asymptomatic but up risk with smoking
PiZZ: significant risk for panacinar emphysema and cirrhosis

220
Q

What type of T-cells mediate asthma initiation?

A

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
Q

What eosinophilic product is a major mediator of asthma inflammation (later stage)

A

Major basic protein

222
Q

What type of lung problem does allergic bronchopulmonary aspergillosis cause?

A

Bronchiectasis

223
Q

What induces fibrosis in IPF? What does it initially look like on CT? Eventually?

A

TGF-beta from injured pneumocytes

Initially is sub pleural, patchy fibrosis that eventually becomes diffuse with “honeycomb” lung

224
Q

Asteroid/stellate bodies in giant cells?

A

Sarcoid

225
Q

What if you see salivary and lacrimal gland destruction along with non-caseating granulomas in hilum?

A

Sarcoid, be careful with Sjogrens

226
Q

Plexiform lesions in lungs

A

Pulmonary hypertension

227
Q

What is the relation of maternal diabetes to NRDS?

A

Insulin decreases surfactant production

228
Q

Why do you see necrotizing entercolitis and PDA with NRDS?

A

NRDS–> hypoxemia–> causes Nec.E and PDA

229
Q

What is the second most frequent cause of lung cancer in US? How are we exposed?

A

Radon exposure, uranium in the soil decays to radon (ex. can be in basements)

230
Q

Tumor composed of lung tissue and cartilage that may be calcified (in lung)

A

Bronchial hamartoma

231
Q

What type of lung cancer is not amenable to surgical resection?

A

Small cell carcinoma

232
Q

What cancer often forms a polyp-like mass in the bronchus?

A

Carcinoid tumor

233
Q

What is a unique site of metastasis for lung tumors?

A

Adrenals

234
Q

What serves as the interim kidney for the first semester? What forms the final kidney?

A

Interim: mesonephros
Final: metanephros

235
Q

What parts of the kidney does the ureteric bud form? What layer is the rest of it from?

A

From ureter back to collecting ducts. The rest is formed by metanephric mesenchyme (glomerulus through DCT)

236
Q

How does the metanephros part of the kidney form?

A

Induced by infiltration by the ureteric bud

237
Q

What is a multicystic dysplastic kidney?

A

Nonfunctional kidney consisting of cysts and connective tissue

238
Q

What is the pattern of blood flow through the kidney?

A

segmental, intralobar, arcuate, intralobular, afferent arteriole, glomerulus, efferent arteriole,peritubular capillaries/vasa recta

239
Q

What is the JG apparatus?

A

Modified SM cells between the DCT and afferent arteriole that is responsible for renin secretion

240
Q

What substance can be used to measure plasma volume? Extracellular volume?

A

Albumin for plasma volume, inulin/mannitol for extracellular volume

241
Q

How much water is in each body compartment?

A

60% total body water, 40% ICF, 20% ECF

Plasma=25% ECF

242
Q

Does creatinine clearance over or underestimate GFR? What about PAH with total renal plasma flow?

A

Creatinine overestimates slightly (some is secreted)

PAH underestimates slightly (some gets through)

243
Q

Where do prostaglandins and ang. II have their effects on the renal vasculature?

A

Ang. II constricts the efferent arteriole while PGs dilate the afferent arteriole

244
Q

How is glucose reabsorbed in the PCT?

A

Na+/glucose cotransport

245
Q

How does PCT reabsorption affect tonicity?

A

It is isotonic

246
Q

Where does PTH act to secrete phosphate and resorb calcium?

A

Phosphate in the PCT, Calcium in the DCT (e.g. thiazides)

247
Q

What does angiotensin II do at the PCT?

A

Stimulates Na+/H+ exchange causing increased Na+, H20 and HCO3- reabsorption

248
Q

How does the TALH cause Mg2+ and Ca2+ reabsorption?

A

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
Q

Is the TALH permeable to water? DCT?

A

No and no

250
Q

Where does urine become fully dilute?

A

Early DCT

251
Q

Where is the most ANYTHING reabsorbed? Potentially besides Calcium and magnesium

A

PCT

252
Q

How do thiazides cause calcium reabsorption?

A

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
Q

What is fanconi syndrome? What notable drugs can cause it?

A

Generalized reabsorption defect in the PCT, expired tetracyclines

254
Q

Locations of Bartter, Gitelman, Liddle Syndrome? Inheritance?

A 
Alphabetic order
Bartter: TALH (~loop diuretics)
Gitelman: DCT (~thiazides)
Liddle: Collecting ducts (~aldosterone, ACTIVATING)
All are AR except Liddle is AD
255
Q

What is syndrome of apparent mineralocorticoid excess? What else can cause it?

A

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
Q

What happens to inulin concentration in the PCT along the length of it?

A

It increases in concentration but not amount due to water reabsorption

257
Q

What three stimuli cause increased renin secretion?

A 
decreased BP (JG cells)
decreased Na+ delivery to DCT (macula densa)
Beta-1 sympathetic activation (sympathetic tone)
258
Q

Why do you not really see reflex bradycardia with angiotensin II?

A

It limits baroreceptor function

259
Q

Where is Epo released from?

A

Interstitial cells in peritubular capillary bed

260
Q

What does dopamine do to kidney function?

A

Secreted by PCT cells and promotes natriuresis.
Low dose: vasodilates everything in kidney
High dose: vasoconstrictor

261
Q

Abdominal pain, kidney stones and mental disturbances?

A

Hypercalcemia

262
Q

How do you calculate anion gap? What is normal?

A

Na-(HCO3+Cl), normal is 8-12

263
Q

What should you check for in metabolic alkalosis?

A

Chloride

264
Q

What is RTA type 1? Type 2? Type 4? Which is hyperkalemia?

A

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
Q

In which RTA type is urine pH increased above 5.5?

A

Type 1, alpha intercalated cells are normally responsible for acidifying the urine

266
Q

What causes type II RTA?

A

Fanconi syndrome and CAII inhibitors

267
Q

If there are casts in the urine, where must the damage be?

A

Glomerulus or renal tubules

268
Q

As nephrotic means protein, what does nephritic mean?

A

Inflammation–> hematuria

269
Q

Starry sky granular appearance on IF? Also called lumpy-bumpy.

A

PSGN

270
Q

What complement protein is associated with PSGN?

A

C3, thus serum C3 is decreased

271
Q

Where are the immune complexes deposited in PSGN?

A

Subepithelial

272
Q

What is the severe renal problem associated with lupus? Characteristic LM finding?

A

Diffuse proliferative glomerulonephritis.

Wire looping of capillaries

273
Q

Episodic gross hematuria that occurs concurrently with respiratory or GI tract infections

A

IgA nephropathy (Berger disease, note NOT BUERGER disease)

274
Q

Alport syndrome inheritance?

A

X-linked dominant

275
Q

MPGN type I has what appearance on microscopy? What is type I associated with? Type II?

A

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
Q

What are the clinically significant effects of protein loss in nephrotic syndrome?

A

1) AT III loss–> hypercoagulability

2) Loss of Ig–> increased infection risk

277
Q

What EM appearance do you see with membranous nephropathy (membranous glomerulonephritis)

A

Spike and dome appearance with subepithelial deposits

278
Q

What antibodies are associated with Membranous Nephropathy?

A

Antibodies to Phospholipase A2 receptor

279
Q

What is the most common kidney stone? Serum and urine findings?

A

Calcium oxalate stone in patient with hypercalciuria and normocalemia

280
Q

What two kidney stones precipitate in low pH? High pH?

A

Low: uric acid and cystine
High: Ammonium magnesium phosphate, calcium phosphate

281
Q

Which kidney stones are radiolucent?

A

Uric acid and cystine

282
Q

What causes calcium oxalate stones?

A

Hypocitraturia

283
Q

What do you suspect with a positive sodium cyanide nitroprusside test?

A

Cystine stones

284
Q

Dilation of renal pelvis and calyces

A

Hydronephrosis

285
Q

Well circumscribed kidney mass with central scar and large eosinophilic cells. Tx? What cells does it arise from?

A

Renal oncocytoma, often resected to exclude malignancy

Arises from collecting duct cells

286
Q

What cell does renal cell carcinoma arise from?

A

PCT cells

287
Q

What is WAGR complex?

A

Wilms tumor, Aniridia, GU malformations, Retardation

288
Q

Chromosome for wilms tumor?

A

11

289
Q

What is suggested by painless hematuria with no casts?

A

Bladder cancer

290
Q

What can cause squamous cell carcinoma of the bladder?

A

Chronic irritation–> metaplasia–> cancer

E.g. schistosoma haematobium

291
Q

Are there systemic signs with urinary tract infections?

A

No, usually only with pyelonephritis

292
Q

What does positive urine nitrites indicate?

A

Gram negative bacteria

293
Q

WBC casts and WBCs in urine suggests what?

A

Acute pyelonephritis

294
Q

Are the glomeruli/vessels or cortex more affected with acute pyelonephritis?

A

Cortex, glomerulus mostly spared

295
Q

What does thyroidization of the kidney indicate?

A

Chronic pyelonephritis (usually means something like vesicoureteral reflux or chronically obstructing stones)

296
Q

What is renal osteodystrophy? What area of bones is affected?

A

Hypocalcemia, hyperphosphatemia, and failure of vitamin D hydroxylation with chronic renal disease. Causes subperiosteal thinning of bones

297
Q

Serum BUN/Cr in prerenal vs. intrinsic renal failure

A

Prerenal: BUN/Cr >20
Intrinsic: BUN/Cr

298
Q

What type of dyslipidemia is a consequence of renal failure?

A

Triglyceridemia

299
Q

Pyuria of eosinophils and azotemia?

A

Acute interstitial nephritis

300
Q

What is the effect on potassium levels of the maintenance and recovery phase of ATN?

A

Maintenance: risk of hyperkalemia (kidney can’t get rid of it)
Recovery: risk of hypokalemia (kidney is working well again)

301
Q

Gross hematuria and proteinuria with sloughing of necrotic renal papillae

A

Renal papillary necrosis

302
Q

Chromosomes for PKD1 and PKD2 in adult polycyctic kidney disease?

A

16, 4 respectively

303
Q

What is dilated in AR polycystic kidney disease? Association?

A

Collecting ducts, hepatic fibrosis and hepatic cysts

304
Q

What diuretic is CI in anuria?

A

Mannitol

305
Q

Effect of loop diuretics on afferent arteriole?

A

Stimulate PGE release and thus afferent vasodilation

306
Q

What is ethacrynic acid?

A

Non-sulfa loop inhibitor. More ototoxic

307
Q

What is metolazone?

A

Thiazide diuretic

308
Q

What are the effects of thiazides on glucose, lipids, uric acid?

A

hyper all of them

309
Q

What does inhibition of ACE due to bradykinin?

A

Prevents inactivation (it is a potent vasodilator)

310
Q

What is aliskiren?

A

Direct renin inhibitor (blocks angiotensinogen conversion to Ang. I)

311
Q

How is dysplastic kidney inherited?

A

It isn’t, risk in subsequent pregnancy is very low

312
Q

Baby with portal hypertension?

A

Polycystic kidney disease

313
Q

ARF with oxalate crystals in the urine?

A

Ethylene glycol toxicity

314
Q

What lipid changes are seen with nephrotic syndrome?

A

Hyperlipidemia and hypercholesterolemia to keep the osmolarity of the serum high

315
Q

What might MCD be associated with rarely?

A

Hodgkin lymphoma

316
Q

Which nephrotic syndrome will respond to steroids?

A

MCD

317
Q

What nephritic and nephrotic syndromes are associated with SLE?

A

Nephrotic: Membranous nephropathy (caucasian one)
Nephritis: DPGN (severe)

318
Q

Which kidney pathologies are associated with HBV and HCV?

A

Membranous nephropathy and membranoproliferative glomerulonephritis (type 1: tram tracks)

319
Q

What is C3 nephritic factor? What is it associated with?

A

It is an autoantibody that stabilizes c3 convertase causing over activation of complement. Associated with dense deposit disease (Type II MPGN)

320
Q

Why do you see microalbuminuria in diabetes?

A

Preferential hyaline arteriolosclerosis of the efferent arteriole causes hyper filtration (higher filtration pressure)

321
Q

What are crescents composed of in RPGN?

A

Fibrin and macrophages

322
Q

What type of nephritic syndrome does lupus cause? Broad class and specific?

A

DPGN which is crescentic glomerulonephritis

323
Q

Can’t pee, can’t see, can’t hear a buzzing bee

A

Alport syndrome

324
Q

Scarring at upper and lower kidney poles is characteristic of what?

A

Vesicoureteral reflux

325
Q

What kidney stone is most commonly seen in children?

A

Cystine stones

326
Q

What occurs with cystic development during dialysis?

A

Increased risk for renal cell carcinoma

327
Q

What is angiomyolipoma associated with?

A

Tuberous sclerosis

328
Q

How does VHL mutation cause renal carcinoma?

A

VHL leads to increased IGF-1 and increased HIF transcription factor which increases VEGF and PDGF

329
Q

Major risk factor for sporadic renal carcinoma?

A

Cigarette smoke

330
Q

Popular syndrome with Wilms tumor?

A

WAGR: Wilms, Aniridia, Genital abnormalities and Retardation

331
Q

What are the two pathways to develop urothelial carcinoma? Describe the differences

A

1) Flat: starts high grade, flat development, EARLY p53 mutation
2) Papillary: low grade to high grade to invasion

332
Q

In what circumstance can an adenocarcinoma arise in the lower urinary tract?

A

urachal remnant

333
Q

What is the life span of RBCs and platelets?

A

RBC: 120 days
Platelets: 8-10 days

334
Q

Why are reticulocytes blue on Wright-Giemsa stain?

A

residual ribosomal RNA

335
Q

Where are platelets stored? How much?

A

1/3 stored in spleen

336
Q

Where do you find collagenase, lysozyme and lactoferrin? B-glucuronidase?

A

First three specific granules of neutrophils, last one in azurophilic granules of neutrophils

337
Q

What about macrophages initiates septic shock?

A

Lipid A from bacterial LPS binds CD14 on macrophages to initiate septic shock

338
Q

What cells contain heparin and histamine?

A

Basophils and mast cells

339
Q

When do you see basophilia?

A

CML

340
Q

What MHC can B cells use to function as an APC?

A

MHC II- important for class switching

341
Q

What is “the Hoff” in plasma cells?

A

Large golgi apparatus

342
Q

What do HbS and HbC replace glutamate with?

A

Valine and lysine (+) respectively

343
Q

What is the typical hemophilia inheritance pattern? Exception?

A

XR, except hemophilia C (factor XI) is AR

344
Q

How does tPA work?

A

Causes plasminogen to be activated to plasmin which cleaves fibrin clots

345
Q

What enzyme is required to activate vitamin K to be a cofactor for gamma-glutamyl carboxylase?

A

Epoxide reductase (warfarin targets this VKOR)

346
Q

What clotting factor does vWF carry and protect? Which factor dies first?

A

VIII, VII

347
Q

What mediates the transient vasoconstriction following endothelial damage?

A

Endothelin

348
Q

What substance induces GPIIb/IIIa expression on platelets?

A

ADP

349
Q

What is the MOA of clopidogrel? Ticlopidine? Abciximab? Tirofiban?

A

First two are ADP receptor blockers and the second two are direct GPIIb/IIIa inhibitors

350
Q

What causes acanthocytes? Echinocytes? Difference?

A

Acanthocyte: abetalipoproteinemia
Echinocyte: more uniform and smaller projections
Echinocytes have projections that are more uniform and smaller

351
Q

When do you see bite cells?

A

G6PD deficiency (post heinz body)

352
Q

Where is the excess iron stored in ringed sideroblasts?

A

Mitochondria

353
Q

What is a heinz body? Howell-Jolly body?

A
  • Hb precipitates

- nuclear remnants in patients with hyposplenia/asplenia

354
Q

What class of anemia does lead poisoning cause?

A

Microcytic (copper deficiency does too)

355
Q

Spoon nails are associated with what?

A

Iron deficiency anemia

356
Q

IDA, esophageal webs, dysphagia?

A

Plummer-Vinson Syndrome

357
Q

What is HBH, Hb Barts?

A

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
Q

Who is more likely to have a cis deletion of the alpha thalasemia genes?

A

Asians

359
Q

What is the genetic defect in beta thalassemia?

A

Point mutations in splice sites and promoter sequences

360
Q

What two enzymes are inhibited by lead poisoning?

A

ALAD and ferrelchelotase

361
Q

Where do you see lead lines?

A

Gingivae and metaphyses of long bones

362
Q

What neuro signs do you see with lead poisoning? Treatment (what about kids?)

A

Foot and wrist drop

Dimercaprol and EDTA are 1st line, succimer used for chelation in kids

363
Q

Defect in sideroblastic anemia? Causes?

A

X-linked defect in ALAS. Alcohol, lead, vitamin B6 deficiency, copper deficiency, INH

364
Q

What is the treatment for sideroblastic anemia?

A

Vitamin B6

365
Q

Defect in orotic aciduria? Blood findings?

A

Decreased UMP synthase, megaloblastic anemia refractory to folate and cobalamin

366
Q

Difference in Diamond Blackfan anemia and fanconi anemia?

A

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
Q

What causes PNH? Treatment?

A

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
Q

What causes warm hemolytic anemia? Cold?

A

Warm: IgG–> SLE and CLL
Cold: IgM–> mycoplasma and mono

369
Q

What do corticosteroids do to WBC levels? how?

A

Increased neutrophils: impaired adhesion and migration out of blood
Decreased esoinophils: sequestered in LN
Decreased lymphocytes: apoptosis

370
Q

Which step of heme synthesis requires vitamin B6 as a cofactor? What inhibits this step?

A

ALAS, heme/glucose

371
Q

AIP and PCT enzymes?

A

AIP: PBGD
PCT: UROD

372
Q

How does iron cause cell damage?

A

Peroxidation of membrane lipids

373
Q

What causes immune thrombocytopenia?

A

Anti-GPIIb/IIIa antibodies (commonly due to viral illness)

374
Q

What causes TTP? Pentad of sx?

A 
ADAMTS13 deficiency (cleaves vWF multimers)
Neuro sx, thrombocytopenia, hemolytic anemia, renal failure, fever
375
Q

What is the inheritance of and treatment for vWF deficiency?

A

AD, treat with desmopressin

376
Q

How do you detect antithrombin deficiency?

A

No direct effects, but causes a decrease in the PTT change with heparin administration.

377
Q

What factors do proteins C and S target?

A

Five and eight

378
Q

What electrolyte abnormalities may be caused by blood transfusion?

A

Hyperkalemia and hypocalcemia (citrate)

379
Q

Which lymphoma type displays: noncontiguous spread? EBV association? Good prognosis?

A

Non-hodgkin, hodgkin, hodgkin

380
Q

Cell markers for reed-sternberg cells?

A

CD 15 and 30

381
Q

Cancer with lytic bone lesions and hypercalcemia besides multiple myeloma

A

Adult T-Cell Lymphoma

382
Q

Atypical CD4+ cells with “cerebriform” nuclei and skin patches/plaques. What can it progress to?

A

Mycosis fungoides–> Sezary syndrome (T-cell leukemia)

383
Q

Most common non-Hodgkin lymphoma in adults

A

DLBCL

384
Q

Starry sky appearance? Where is the sporadic form? Mutation?

A

Burkitt, pelvis or abdomen, t(8:14) c-myc with Ig heavy chain

385
Q

What might an eosinophilic protein in the urine make you consider? Blood finding?

A

Multiple Myeloma (Bence-Jones), Rouleaux formation

386
Q

Neutrophils with bilobed nuclei: what are they and when do you see them?

A

Pseudo-Pegler-Huet anomaly and after chemotherapy

387
Q

Indolent, waxing and waning lymphoma. Mutation?

A

Follicular lymphoma (BCL-2 t(14:18))

388
Q

What does TdT+ mean? CD10+?

A

Lymphoblast (T or B cells), marker of pre B-cells

389
Q

Which cancers are associated with Down syndrome?

A

B-ALL, AML

390
Q

What are two odd places that ALL may spread to that require specific treatment? Mutation with good prognosis?

A

CNS and testes. t(12;21)

391
Q

What do you suspect with CD20+, CD5+ cells? What type of cells are they? Specific finding?

A

CLL, B-cells, Smudge cells

392
Q

Whats the difference with smudge and hairy cells on appearance?

A

Smudge cells are odd nuclei, hairy cells are talking about the cytoplasm

393
Q

Hairy cell leukemia: Stains positive for what? Treatment?

A

TRAP+, Cladribine

394
Q

What is a specific cell finding for AML? What is it? Translation? Treatment? Common presentation

A

Auer rods (myeloperoxidase), t(15;17), ATRA for treatment. Commonly presents as DIC

395
Q

Basophilia suggests what?

A

CML

396
Q

How do you differentiate CML neutrophils with a leukemoid reaction?

A

1) Low LAP as a result of low activity in malignant neutrophils
2) Increased basophils
3) Mutation (t(9;22)= BCR-ABL

397
Q

Lytic bone lesions and skin rash or recurrent otitis media with mass involving mastoid bone. What do cells express?

A

Langerhans cell histiocytosis, S-100

398
Q

Mutation with myeloproliferative disorders?

A

V617F JAK2

399
Q

Severe, burning pain and red-blue coloration in extremities?

A

Erythromyalgia- rare but classic with PV, also possible with ET

400
Q

What cancers can cause inappropriate absolute polycythemia as a paraneoplastic syndrome?

A

Renal cell carcinoma, hepatocellular carcinoma

401
Q

What are the EPO levels in PV?

A

Low, feedback suppresses it

402
Q

What does heparin work on?

A

Antithrombin III which lowers the activity of thrombin and factor Xa

403
Q

Which is used in pregnancy, heparin or warfarin?

A

Heparin

404
Q

How do you identify LMWH drugs? What do they do?

A

“-parin” is in name but not heparin. More specific for factor Xa

405
Q

What antibody is associated with HIT?

A

IgG against platelet factor 4 (PF4)

406
Q

What is bivalirudin?

A

Direct thrombin inhibitor derived from leech anticoagulant

407
Q

What is streptokinase?

A

Thrombolytic

408
Q

What is ticlopidine? Side effect?

A

ADP receptor blocker, neutropenia

409
Q

What are cilostazol and dipyridamole?

A

Phosphodiesterase III inhibitors causing vasodilation and inhibition of platelet aggregation

410
Q

What are eptifibatide and tirofiban?

A

GPIIb/IIIa inhibitors (“fib”), also abciximab

411
Q

What activates azathioprine and 6-MP? What do they do?

A

HGPRT, purine analogs decreasing de novo purine synthesis

412
Q

Side effects of cladribine (3)?

A

Myelosuppression, nephrotoxicity, neurotoxicity

413
Q

What enzyme does 5-FU inhibit? How does leucovorin affect it?

A

Thymidylate synthase by covalently complexing folic acid. Leucovorin enhances the activity

414
Q

What does methotrexate do? How does leucovorin affect it?

A

Dihydrofolate reductase inhibitor that is reversed by leucovorin.

415
Q

What drug specifically acts in the G2 phase?

A

Bleomycin (free radical formation)

416
Q

What drug is often used in childhood tumors?

A

Dactinomycin

417
Q

What is the side effect of doxorubicin? What prevents it?

A

Cardiotoxicity (dilated cardiomyopathy) which can be prevented by dexrazoxane

418
Q

How does cyclophosphamide work? Side effects? What prevents them?

A

Cross-links DNA at guanine N7.

Hemorrhagic cystitis prevented by mesna or NAC

419
Q

What drugs are often used for brain tumors?

A

Nitrosoureas (-ustine, streptozocin)

420
Q

How do you prevent cisplatin toxicity?

A

Prevent nephrotoxicity with amifostine and chloride diuresis

421
Q

What is the MOA of irinotecan?

A

Inhibits topoisomerase I

422
Q

What is the MOA of hydroxyurea?

A

Inhibits ribonucleotide reductase (DNA synthesis)

423
Q

What is bevacizumab?Erlotinib? Cetuximab? Imatinib?

A

Monoclonal antibody against

VEGF, EGFR tyrosine kinase inhibitor, EGFR mab, tyrosine kinase inhibitor of BCR-ABL

424
Q

What antibody increases the risk of PML?

A

Rituximab (CD 20)

425
Q

What is Vemurafenib?

A

inhibit V600E BRAF mutation (V600E MUtated, bRAF INHIBition)

426
Q

Toxicity of trastuzumab?

A

Cardiotoxicity (“heartceptin”)

427
Q

What does vWF bind to besides platelets? Where does it come from?

A

Exposed subendothelial collagen. Weibel-Palade bodies and alpha granules of platelets

428
Q

What is ITP caused by? What fixes it?

A

Plasma cells in the spleen produce IgG against platelet antigens like GPIIb/IIIa. Then spleen removes antibody bound platelets. Treatment is splenectomy

429
Q

What is the effect of uremia on platelets?

A

Impairs both adhesion and aggregation

430
Q

What activates factors XII and VII?

A

XII: sub endothelial collagen
VII: Tissue thromboplastin

431
Q

What do you suspect with increased PTT that does not correct upon mixing normal plasma?

A

Coagulation factor inhibitor

432
Q

Treatment for vWF disease? Inheritance?

A

AD, desmopressin which increase W-P body vWF release

433
Q

What coagulation time can be watched for liver failure?

A

PT (factor VII is the first to go)

434
Q

Why might you see thrombosis in HIT?

A

Because destroyed platelet fragments may activate remaining platelets (sort of paradoxical)

435
Q

Name two odd things that can cause DIC. One is a cancer and the other is a toxin

A

Adenocarcinoma: mucin activates coagulation

Rattlesnake bite: venom activates coagulation

436
Q

How does plasmin stop clotting? What stops plasmin?

A

Plasmin: 1) cleaves fibrin and fibrinogen
2) Destroys coagulation factors
3) Blocks platelet aggregation
It is stopped by alpha 2 antiplasmin

437
Q

Name two things that can cause a disorder of fibrinolysis (plasmin over activation)

A

1) Radical prostatectomy: urokinase release activates plasmin
2) Cirrhosis: reduced alpha 2 antiplasmin production

438
Q

Increased PT/PTT, increased bleeding time, increased fibrinogen split products. What is the platelet count? What is the treatment?

A

This is plasmin over activation (no D-dimer, increased FIBRINOGEN split). Normal platelet count. Treat with aminocaproic acid which blocks plasminogen activation

439
Q

How do B12 and folate deficiency increase thrombosis risk?

A

They increase homocysteine levels by preventing conversion to methionine. Homocysteine damages endothelial cells and promotes thrombosis

440
Q

What enzyme takes homocysteine to cystathionine?

A

cystathionine beta synthase (CBS)

441
Q

Dyspnea and petichiae on skin?

A

Fat embolus (neuro sx too)

442
Q

Squamous cells and debris in an embolus

A

Amniotic fluid embolus

443
Q

What arteries provide dual supply to the lungs?

A

Pulmonary arteries and bronchial arteries

444
Q

What is the general reason for microcytic anemia?

A

Decreased hemoglobin production–> extra division of RBC to maintain Hb concentration

445
Q

What is heme composed of?

A

Iron and protoporphyrin

446
Q

Where is iron absorbed?

A

Duodenum

447
Q

Most likely causes of IDA in developing world elderly population?

A

Hookworms: Ancylostoma duodenale and Necator americanus

448
Q

What is increased in erythrocytes in IDA?

A

Free erythrocyte protoporphyrin (FEP)

449
Q

What causes sideroblastic anemia?

A

Decreased protoporphyrin synthesis (e.g. lead poisoning)

450
Q

What step in protoporphyrin synthesis requires B6 as a cofactor?

A

ALAS

451
Q

Where is iron trapped in sideroblastic RBCs? Why?

A

Iron is in the mitochondria because this is where it would normally join protoporphyrin to make heme

452
Q

What drug can cause sideroblastic anemia? Habit? How?

A

INH causes B6 deficiency. Alcohol is a mitochondrial poison.

453
Q

What is the state of iron in siderblastic anemia?

A

Iron overloaded state

454
Q

Are the globin chains made in thalassemia defective?

A

No, just a decrease in synthesis

455
Q

How many alpha thalassemia genes are there? Chromosome? What about beta?

A

Four alpha on chromosome 16

2 beta on chromosome 11

456
Q

Which thalassemia is due to deletions and which to mutations?

A

Alpha is primarily due to deletions while beta is due to mutations

457
Q

Which thalassemia is associated with extra medullary hematopoiesis?

A

Alpha

458
Q

What causes macrocytic anemia generally?

A

Lack of DNA means RBCs cannot keep dividing and stay larger as a result

459
Q

What is pernicious anemia? What deficiency is caused?

A

Autoimmune destruction of parietal cells leading to intrinsic factor and thus B12 deficiency

460
Q

What is B12 converting that leads to SCD with deficiency? What step forms the MMA?

A

methylmalonylic acid to succinyl CoA

Propionyl CoA to MMA via Propionyl CoA carboxylase

461
Q

What causes hemosiderinuria?

A

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
Q

Most common cause of death in adult sickle cell patients

A

Acute Chest Syndrome (vaso-occlusion in pulmonary microcirculation)

463
Q

Where does sickling still occur in sickle cell trait?

A

Renal medulla (extreme hypoxia and hypertonicity)

464
Q

What does the metabisulfate screen do?

A

Any cells with any amount of HbS sickle (trait and disease)

465
Q

Why does PNH happen at night?

A

Mild respiratory acidosis with shallow breathing at night activates complement

466
Q

What is the main cause of death in PNH? What cancer can develop and why?

A

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
Q

Hemoglobinuria and back pain several hours after getting dapsone tx?

A

G6PD deficiency. Back pain since Hb is nephrotoxic

468
Q

What is the cell mark on a heatopoietic stem cell that leads to myeloid and lymphoid cell lines?

A

CD34+

469
Q

Why would severe infection cause leukopenia?

A

Increased movement of neutrophils into tissues

470
Q

What is the most sensitive cell in the body to radiation?

A

Lymphocytes

471
Q

What cell line leads to erythroblasts?

A

Myeloid stem cell

472
Q

Decrease in what receptor indicates immature leukocytes?

A

Fc receptors (CD 16)

473
Q

What is a notable case where you see eosinophilia? Basophilia?

A

Eosinophilia in Hodgkins lymphoma, basophilia in CML

474
Q

What bacteria can produce lymphocytic leukocytosis?

A

Bordatella pertussis

475
Q

What causes LAD in mononucleosis? Splenomegaly?

A

T-cell (CD8+) hyperplasia in lymph node paracortex and Periarterial lymphatic sheath (PALS)

476
Q

What is TdT? When do you see it?

A

Lymphoblasts and it is a DNA polymerase

477
Q

Which tumor is with down syndrome before 5 years old? After?

A

Before is AM (megakaryoblastic)L, After is ALL (LL= later later)

478
Q

Leukemia that classically infiltrates gums?

A

Acute monocytic leukemia

479
Q

What type of leukemia might myelodysplastic syndromes develop into?

A

AML

480
Q

What do CLL cells express in regards to cell markers? What type of cells are they?

A

B cells that coexpress CD5 (normally on T cells) and CD20

481
Q

Why do you get splenomegaly in Hairy Cell Leukemia?

A

They accumulate in the RED pulp (this is odd because WBCs normally live in white pulp)

482
Q

What if you see lytic lesions, HSM, hypercalcemia and a rash?

A

ATLL (HTLV associated)

483
Q

Aggregates of neoplastic CD4+ cells in the epidermis?

A

Pautrier micro abscesses with mycosis fungiodes

484
Q

What can CML progress to? Why?

A

AML or ALL because the mutation is in a pluripotent stem cell

485
Q

Does ET progress to marrow fibrosis or leukemia? Cause gout?

A

No and no, unlike other MPDs

486
Q

What proliferates in myelofibrosis?

A

Megakaryocytets which produce excess PDGF and cause fibrosis of the marrow

487
Q

In a lymph node draining a tissue with cancer, what area is hyperplastic?

A

Medulla (sinus histiocytes)

488
Q

What three diseases are associated with marginal zone lymphoma? Another name of it? When is the marginal zone present?

A

Only present in chronic inflammation

H. pylori (MALToma), Hashimoto, Sjogren

489
Q

Most common HL subtype? Best prognosis? Eosinophils?

A

Nodular sclerosis- most common
Lymphocyte-rich- best prognosis
Mixed cellularity- eosinophils

490
Q

What IL might be high with multiple myeloma?

A

IL-6 stimulates plasma cell growth and Ig production

491
Q

How does multiple myeloma cause bone lesions?

A

Activates RANK receptor on osteoclasts

492
Q

What markers are positive in LCH?

A

CD1a+ and S100+

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