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Hemeonc MSK Flashcards

1
Q

Anisocytosis

A

varying sizes

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2
Q

Poikilocytosis

A

varying shapes

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3
Q

thrombocytopenia/platelet dysfunction results in

A

petechiae

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4
Q

Cell surface marker for Macrophages?

A

MC14

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5
Q

What causes eosinophilia?

A 
DNAAACP
Drugs
Neoplasms
Atopy/allergy
Addisons
Acute interstitial neph
Collagen vascular dx
Parasites- helminths
- Churgg Strauss
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6
Q

funtion of a mast cell?

A

binds to the Fc portion of IgE to membrane

- cross linking of IgE causes degranulation releasing histamine, heparin and eosinophil chemotactic factors

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7
Q

only APC that can activate NK cells?

A

Dendritic cells- acts as a linke between innate and adaptive immune system

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8
Q

CD28

A

marker of T cells, necessary for t cell activation

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9
Q

what enzyme does warfarin inhibit?

A

epoxide reductase which usually activates vitamin K to make mature clotting factors

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10
Q

Role of kallikrein?

A

activates bradykinin and complement cascade (via activation of plasmin)

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11
Q

C1 esterase inhibitor?

A

inhibits kallikrein > brakykinin, so a deficiency causes a buildup of bradykinin, leading to angioedema

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12
Q

role of plasmin?

A

decreases the fibrin clot

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13
Q

What does the endothelial cell make in terms of thrombogenesis?

A

vWF
thromboplastin
tPA
PGI2

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14
Q

when do you see a acanthocyte?

A

abetalipoproteinemia

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15
Q

When do you see target cells?

A 
HALT
HbC
Asplenia
Liver disease
Thalassemia
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16
Q

> 3.4% HbA2

A

diagnostic confirmation fo B Thal minor

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17
Q

what are the genetics behind beta thal?

A
  • point mutations in splice sites and promoter sequences decreasing B globin synthesis
    (Alpha thal = gene deletions)
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18
Q

crew cut on skull xray, chipmunk facies

A

beta thal major- homozygotes, stop codon, increased HbF

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19
Q

what are the aggregates seen in basophilic stipling?

A

rRNA aggregates

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20
Q

where in the bone does lead target?

A

epiphysis of distal femur and proximal tibia

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21
Q

Pathogenesis of sideroblastic anemia

A

X linked defect in d-ALA synthase gene

- also seen in alcohol, lead and INH deficiency

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22
Q

tx of sideroblastic anemia?

A

pyridoxine- B6 is a cofactor for ALA synthase

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23
Q

findings of intravascular hemolysis

A

decreased haptoglobin
increased LDH
hemoglobinuria

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24
Q

Extravascular hemolysis findings

A

increased LDH

increased UCB > jaundice

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25
Q

What viruses can cause Aplastic anemia?

A

Parvo B19
EBV
HCV
HIV

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26
Q

how does pyruvate kinase deficiency cause anemia?

A

defective pyruvate kinase causes decreased ATP > rigid RBCs

- hemolytic anemia in newborn

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27
Q

whats the defect behidn HbC?

A

glutamic acid to lysine mutation in B globin gene

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28
Q

treatment of PNH?

A

eculizumab

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29
Q

genetics of HbS?

A

substitution of glutamic acid with val in Beta chain

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30
Q

What drug could cause Warm agglutinin autoimmune hemolytic anemia?

A

alpha-methyldopa

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31
Q

What enzymes does Lead inhibit in heme synthesis? what builds up?

A
  1. Ferrochelatase
  2. ALA dehydrogenase
    Buildup: protoporphyrin and delta-ALA
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32
Q

Acute intermittent porphyria: affected enzyme and accumulated substrate

A

Enzyme: porphobilinogen deaminase
Substrate: prophobilinogen, delata-ALA, uroporphyrin

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33
Q

abdominal pain, burgundy colored urine, polyneuropathy, psych disturbances

A

Acute intermittent porphyria

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34
Q

Porphyria cutanea tarda: affected enzyme and accumulated substrate

A

Enzyme: uroporphyrinogen decarboxylase

Accumulated substrate: uroporphyin (tea colored urine)

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35
Q

blistering cutaneous photosensitivty and tea colored urine

A

Porphyria cutanea tarda

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36
Q

what disease is a combination of AIP with photosensitivity?

A

Coroporphyrinogen III oxidase deficiency

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37
Q

what is the treatment for Acute intermittent porphyria?

A

glucose and heme (inhibit ALA synthase)

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38
Q

what are the signs of a platelet disorder versus coagulation disorder?

A

Coagulation: bleeding into joints easy bruising
Platelet: epistaxis, petechiae, purpura

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39
Q

Bernard soulier syndrome

A

decreased GpIb leading to defect in paltelet to vWF adhesion

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40
Q

Glanzmanns thrombasthenia

A

decreased GpIIb/IIIa, defect in platelet to platelet aggregation

  • blood smear shows no platelet clumping
  • normal platelet count
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41
Q

Idiopathic thrombocytopenic purpura

A

anti-GpIIb/IIIa antibodies, splenic macs eat platelet/antibody complex

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42
Q

TTP

A

deficiency of ADAMTS13 (vWF metalloprotease) > decrease of vWF multimers
- neurologic, renal sx, fever, thrombocytopenia, and microangiopathic hemolytic anemia

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43
Q

How is heme degraded?

A

by Heme oxygenase- turning it into CO2 and biliverdin (which gives bruises their green color)

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44
Q

inheritance of vWF disease

A

Autosomal Dominant

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45
Q

tx of vWF disease?

A

give DDAVP (desmopressin) which causes release of vWF stored in endothelium

46
Q

someone bleeding from venous puncture sites

47
Q

prothrombin gene mutation

A

mutation in 3” untranslated region

increases prothrombin > increased venous clots

48
Q

antiphospholipid antibody

A
  • lupus anticoagulant or anticardiolipin antibodies plus one of the following:
  • venous thromboembolism
  • arterial thromboembolism
  • frequent fetal loss
    also increased aPTT
49
Q

how can a blood transfusion cause hypocalcemia?

A
  • citrate is a calcium chelator
50
Q

Reed sternburg cells are CD what?

A

CD30 and CD15: B cell origin

51
Q

what type of hodgkins lymphoma has best prognosis?

A

lymphocyte rich = best prog

lymphocyte depleted = worst prognosis

52
Q

which lymohoma is CD5+?

A

mantle cell

53
Q

Chrom 8:
chrom 11:
chrom 14:
Chrom 18:

A

Chrom 8: c-myc
chrom 11: cyclin D1
chrom 14: Ig heavy chain
Chrom 18: bcl-2

54
Q

Signs of multiple myeloma

A 
CRAB
hyperCalcemia
Renal insufficiency
Anemia
Bone lytic lesions and Bence Jones proteins
55
Q

smudge cells seen on peripheral blood smear

56
Q

treatment of hairy cell leukemia?

A

caldribine- an adenosine analog

57
Q

what happens when auer rods are released in the body?

58
Q

what can CML change into?

A

AML or ALLL

59
Q

lytic bone lesion + skin rash, what is it and what does it express?

A

langerhans cell histiocytosis

- express S-100 and CD1a (S100 = neural crest cell origin)

60
Q

intense itching after a shower

A

polycythemia vera

61
Q

What is the inheritance of BRCA-1? what is it and how is it mutated in cancer?

A

autosomal dominant

- it is a tumor suppressor, performs DNA repair and transcription

62
Q

what is a possible consequence of ill fitting crutches?

A

radial nerve issues

- wrist drop and paralysis of extensors

63
Q

what nerve is responsible for opposition of the thumb?

64
Q

where is the defect in thoracic outlet syndrome?

65
Q

whats the difference between the dorsal and palmar interoseous muscles?

A

dorsal: DABs, abduct
palmar: PADs, adduct

66
Q

what is trandelenburg sign and what nerve is defected?

A
  • contralateral hip drops when standing on ipsilateral leg to side of lesion
  • Superior gluteal nerve
67
Q

when does woven bone occur in adults?

A

after fractures and in Pagets disease

68
Q

osteoclasts are a derivative of what?

A

macs and monocytes

69
Q

inheritance of achondroplasia?

A

autosomal dominent

70
Q

what drug class is contraindicated in osteoporosis?

A

glucocorticoids

71
Q

mutation seen in osteopetrosis?

A

carbonic anhydrase II- which impairs ability of osteoclasts to generate acidic environment necessary for bone resorption

72
Q

what is the only lab abnormality seen in Pagets disease?

A

increased ALP due to increased activity of osteoblasts

73
Q

mosaic bon pattern, long bone chalk stick fractures, increasing hat size

A

Pagets disease

74
Q

sequelae of pagets disease?

A
  • increased BF from AV shunts > high output cardiac failure

- increased risk of osteogenic sarcoma

75
Q

osteoclasts are very large and have up to 100 nuclei

A

pagets disease

76
Q

what is mccune albright syndrome?

A
  • polyostotic fibrous dysplasia characterized by multiple unilateral bone lesions assoc with endocrine abnormalities and cafe au lait spots
77
Q

bone tumor: spindle shaped cells with multinucleated giant cells, double bubble or soap bubble appearance

A

giant cell tumor- osteoclastoma

78
Q

bone tumor: anaplastic small blue cell malignant tumor, onion skin appearance on bone

A

Ewings sarcoma

79
Q

translocation seen in ewings sarcoma?

80
Q

bone tumor- expansile glistening mass within the medullary cavity

A

chondrosarcoma

81
Q

type of hypersensitivity: rheumatoid arthritis

82
Q

what is the specific antibody towards RA?

A

anti-cyclic citrullinated peptide antibody

83
Q

Sjogrens syndrome increases risk for what?

A

B cell lymphoma and dental caries

84
Q

what type of crystal is seen in pseudogout? composition?

A
  • rhomboid crystals, weakly positively birefringent

- made of calcium pyrophosphate

85
Q

what cardiac issue is related to ankylosing spondylitis?

A

aortic regurg

86
Q

Reiter’s syndrome

A
  • post GI or chlamydia infection
  • conjunctivitis
  • urethritis
  • arthritis
    “Can’t see, cant pee, cant climb a tree”
87
Q

Libman Sacks endocarditis

A
  • verrucous wart like sterile vegetations on both sides of valve
  • assoc with SLE
88
Q

cause of death in SLE?

89
Q

In SLE, what nephritis and nephrotic syndromes do u see?

A

Nephritis: Diffuse proliferative glomerulonephritis
Nephrotic: Membranoproliferative

90
Q

Why do SLE pts get false positives on syphilis test?

A

antiphospholipid antibodies which cross react with cardiolipin

91
Q

Characteristic findings in sarcoidosis?

A
  • immune mediated widespread noncaseating granulomas
  • elevated serum ACE levels
  • bilateral hilar adenopathy
  • hypercalcemia: due to elevated 1alpha-hydroxylase mediated vit D activation
92
Q

How do you distinguish sarcoidosis from other disease?

A

High CD4:CD8 ratio

93
Q

what is polymyalgia rheumatica related to?

A

giant cell arteritis

94
Q

Difference in immune response in Polymyositis versus dermatomyositis

A

Polymyositis: CD8 T cells
Dermatomyositis: CD4 T cells

95
Q

what is the most likely cause of death in scleroderma?

A

pulmonary!

96
Q

CREST scleroderma

A 
Calcinosis
Raynauds
Esophageal dysmotility
Sclerodactyly
Terlangiectasia
- assoc with anticentromere antibody
97
Q

acantholysis

A

separation of epidermal cells- pemphigus vulgaris

98
Q

What do you see on immunofluorescence of pemphigus vulgaris?

A

potentially fatal IgG antibodies around epidermal cells in a reticular or netlike pattern

99
Q

Pathophys of pemphigus vulgaris?

A
  • potentially fatal autoimmune skin disorder with IgG antibody against desmoglein 3
  • positive nikolsky’s sign (separation of skin upon manual stroking)
100
Q

difference between pemphigus vulgarus and bullous pemphigoid

A

BP: autoimmune disodrer with IgG antibody against hemidesmosomes
- spares oral mucosa
PV: involves oral mucosa, IgG against desmoglein 3

101
Q

what is dermatitis herpetiformis?

A

deposits of IgA at the tips of dermal papillae

- assoc with celiac disease

102
Q

What are the bugs and drugs assoc with Erythema multiforme?

A

Bugs: mycoplasma pneumonia and HSV
Drugs: sulfa drugs, B lactams, phenytoin

103
Q

What is lichen planus? assoc?

A
  • Pruritic, purple, polygonal planar papules and plagues
  • sawtooth infiltrate of lymphocytes at the dermal-epidermal junction
  • assoc with HepC
104
Q

sawtooth infiltrate of lymphocytes at dermal-epidermal junction

A

lihen planus

105
Q

tumor marker of melanoma

106
Q

mutation seen in melanoma

A

BRAF kinase- activating

107
Q

Vemurafenib

A

a BRAF kinase inhibitor- used in melanoma

108
Q

What happens when antibodies bind to their nicotinic ACh receptors in MG?

A

-results in receptors internalization and degradation by complement fixation

109
Q

which artery runs with the radial nerve?

A

deep brachial artery

110
Q

What does the FAS gene do?

A

it deletes clones of autoreactive T lymphocytes

- thought to be involved in pathogenesis of SLE

FA notes (11 decks)

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