Hemeonc MSK Flashcards
(110 cards)
1
Q
Anisocytosis
A
varying sizes
2
Q
Poikilocytosis
A
varying shapes
3
Q
thrombocytopenia/platelet dysfunction results in
A
petechiae
4
Q
Cell surface marker for Macrophages?
A
MC14
5
Q
What causes eosinophilia?
A
DNAAACP Drugs Neoplasms Atopy/allergy Addisons Acute interstitial neph Collagen vascular dx Parasites- helminths - Churgg Strauss
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
7
Q
only APC that can activate NK cells?
A
Dendritic cells- acts as a linke between innate and adaptive immune system
8
Q
CD28
A
marker of T cells, necessary for t cell activation
9
Q
what enzyme does warfarin inhibit?
A
epoxide reductase which usually activates vitamin K to make mature clotting factors
10
Q
Role of kallikrein?
A
activates bradykinin and complement cascade (via activation of plasmin)
11
Q
C1 esterase inhibitor?
A
inhibits kallikrein > brakykinin, so a deficiency causes a buildup of bradykinin, leading to angioedema
12
Q
role of plasmin?
A
decreases the fibrin clot
13
Q
What does the endothelial cell make in terms of thrombogenesis?
A
vWF
thromboplastin
tPA
PGI2
14
Q
when do you see a acanthocyte?
A
abetalipoproteinemia
15
Q
When do you see target cells?
A
HALT HbC Asplenia Liver disease Thalassemia
16
Q
> 3.4% HbA2
A
diagnostic confirmation fo B Thal minor
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)
18
Q
crew cut on skull xray, chipmunk facies
A
beta thal major- homozygotes, stop codon, increased HbF
19
Q
what are the aggregates seen in basophilic stipling?
A
rRNA aggregates
20
Q
where in the bone does lead target?
A
epiphysis of distal femur and proximal tibia
21
Q
Pathogenesis of sideroblastic anemia
A
X linked defect in d-ALA synthase gene
- also seen in alcohol, lead and INH deficiency
22
Q
tx of sideroblastic anemia?
A
pyridoxine- B6 is a cofactor for ALA synthase
23
Q
findings of intravascular hemolysis
A
decreased haptoglobin
increased LDH
hemoglobinuria
24
Q
Extravascular hemolysis findings
A
increased LDH
increased UCB > jaundice
25
What viruses can cause Aplastic anemia?
Parvo B19
EBV
HCV
HIV
26
how does pyruvate kinase deficiency cause anemia?
defective pyruvate kinase causes decreased ATP > rigid RBCs
| - hemolytic anemia in newborn
27
whats the defect behidn HbC?
glutamic acid to lysine mutation in B globin gene
28
treatment of PNH?
eculizumab
29
genetics of HbS?
substitution of glutamic acid with val in Beta chain
30
What drug could cause Warm agglutinin autoimmune hemolytic anemia?
alpha-methyldopa
31
What enzymes does Lead inhibit in heme synthesis? what builds up?
1. Ferrochelatase
2. ALA dehydrogenase
Buildup: protoporphyrin and delta-ALA
32
Acute intermittent porphyria: affected enzyme and accumulated substrate
Enzyme: porphobilinogen deaminase
Substrate: prophobilinogen, delata-ALA, uroporphyrin
33
abdominal pain, burgundy colored urine, polyneuropathy, psych disturbances
Acute intermittent porphyria
34
Porphyria cutanea tarda: affected enzyme and accumulated substrate
Enzyme: uroporphyrinogen decarboxylase
| Accumulated substrate: uroporphyin (tea colored urine)
35
blistering cutaneous photosensitivty and tea colored urine
Porphyria cutanea tarda
36
what disease is a combination of AIP with photosensitivity?
Coroporphyrinogen III oxidase deficiency
37
what is the treatment for Acute intermittent porphyria?
glucose and heme (inhibit ALA synthase)
38
what are the signs of a platelet disorder versus coagulation disorder?
Coagulation: bleeding into joints easy bruising
Platelet: epistaxis, petechiae, purpura
39
Bernard soulier syndrome
decreased GpIb leading to defect in paltelet to vWF adhesion
40
Glanzmanns thrombasthenia
decreased GpIIb/IIIa, defect in platelet to platelet aggregation
- blood smear shows no platelet clumping
- normal platelet count
41
Idiopathic thrombocytopenic purpura
anti-GpIIb/IIIa antibodies, splenic macs eat platelet/antibody complex
42
TTP
deficiency of ADAMTS13 (vWF metalloprotease) > decrease of vWF multimers
- neurologic, renal sx, fever, thrombocytopenia, and microangiopathic hemolytic anemia
43
How is heme degraded?
by Heme oxygenase- turning it into CO2 and biliverdin (which gives bruises their green color)
44
inheritance of vWF disease
Autosomal Dominant
45
tx of vWF disease?
give DDAVP (desmopressin) which causes release of vWF stored in endothelium
46
someone bleeding from venous puncture sites
DIC
47
prothrombin gene mutation
mutation in 3" untranslated region
| increases prothrombin > increased venous clots
48
antiphospholipid antibody
- lupus anticoagulant or anticardiolipin antibodies plus one of the following:
- venous thromboembolism
- arterial thromboembolism
- frequent fetal loss
also increased aPTT
49
how can a blood transfusion cause hypocalcemia?
- citrate is a calcium chelator
50
Reed sternburg cells are CD what?
CD30 and CD15: B cell origin
51
what type of hodgkins lymphoma has best prognosis?
lymphocyte rich = best prog
| lymphocyte depleted = worst prognosis
52
which lymohoma is CD5+?
mantle cell
53
Chrom 8:
chrom 11:
chrom 14:
Chrom 18:
Chrom 8: c-myc
chrom 11: cyclin D1
chrom 14: Ig heavy chain
Chrom 18: bcl-2
54
Signs of multiple myeloma
```
CRAB
hyperCalcemia
Renal insufficiency
Anemia
Bone lytic lesions and Bence Jones proteins
```
55
smudge cells seen on peripheral blood smear
SLL/CLL
56
treatment of hairy cell leukemia?
caldribine- an adenosine analog
57
what happens when auer rods are released in the body?
DIC
58
what can CML change into?
AML or ALLL
59
lytic bone lesion + skin rash, what is it and what does it express?
langerhans cell histiocytosis
| - express S-100 and CD1a (S100 = neural crest cell origin)
60
intense itching after a shower
polycythemia vera
61
What is the inheritance of BRCA-1? what is it and how is it mutated in cancer?
autosomal dominant
| - it is a tumor suppressor, performs DNA repair and transcription
62
what is a possible consequence of ill fitting crutches?
radial nerve issues
| - wrist drop and paralysis of extensors
63
what nerve is responsible for opposition of the thumb?
median
64
where is the defect in thoracic outlet syndrome?
C8 and T1
65
whats the difference between the dorsal and palmar interoseous muscles?
dorsal: DABs, abduct
palmar: PADs, adduct
66
what is trandelenburg sign and what nerve is defected?
- contralateral hip drops when standing on ipsilateral leg to side of lesion
- Superior gluteal nerve
67
when does woven bone occur in adults?
after fractures and in Pagets disease
68
osteoclasts are a derivative of what?
macs and monocytes
69
inheritance of achondroplasia?
autosomal dominent
70
what drug class is contraindicated in osteoporosis?
glucocorticoids
71
mutation seen in osteopetrosis?
carbonic anhydrase II- which impairs ability of osteoclasts to generate acidic environment necessary for bone resorption
72
what is the only lab abnormality seen in Pagets disease?
increased ALP due to increased activity of osteoblasts
73
mosaic bon pattern, long bone chalk stick fractures, increasing hat size
Pagets disease
74
sequelae of pagets disease?
- increased BF from AV shunts > high output cardiac failure
| - increased risk of osteogenic sarcoma
75
osteoclasts are very large and have up to 100 nuclei
pagets disease
76
what is mccune albright syndrome?
- polyostotic fibrous dysplasia characterized by multiple unilateral bone lesions assoc with endocrine abnormalities and cafe au lait spots
77
bone tumor: spindle shaped cells with multinucleated giant cells, double bubble or soap bubble appearance
giant cell tumor- osteoclastoma
78
bone tumor: anaplastic small blue cell malignant tumor, onion skin appearance on bone
Ewings sarcoma
79
translocation seen in ewings sarcoma?
11;22
80
bone tumor- expansile glistening mass within the medullary cavity
chondrosarcoma
81
type of hypersensitivity: rheumatoid arthritis
Type III
82
what is the specific antibody towards RA?
anti-cyclic citrullinated peptide antibody
83
Sjogrens syndrome increases risk for what?
B cell lymphoma and dental caries
84
what type of crystal is seen in pseudogout? composition?
- rhomboid crystals, weakly positively birefringent
| - made of calcium pyrophosphate
85
what cardiac issue is related to ankylosing spondylitis?
aortic regurg
86
Reiter's syndrome
- post GI or chlamydia infection
- conjunctivitis
- urethritis
- arthritis
"Can't see, cant pee, cant climb a tree"
87
Libman Sacks endocarditis
- verrucous wart like sterile vegetations on both sides of valve
- assoc with SLE
88
cause of death in SLE?
nephritis
89
In SLE, what nephritis and nephrotic syndromes do u see?
Nephritis: Diffuse proliferative glomerulonephritis
Nephrotic: Membranoproliferative
90
Why do SLE pts get false positives on syphilis test?
antiphospholipid antibodies which cross react with cardiolipin
91
Characteristic findings in sarcoidosis?
- immune mediated widespread noncaseating granulomas
- elevated serum ACE levels
- bilateral hilar adenopathy
- hypercalcemia: due to elevated 1alpha-hydroxylase mediated vit D activation
92
How do you distinguish sarcoidosis from other disease?
High CD4:CD8 ratio
93
what is polymyalgia rheumatica related to?
giant cell arteritis
94
Difference in immune response in Polymyositis versus dermatomyositis
Polymyositis: CD8 T cells
Dermatomyositis: CD4 T cells
95
what is the most likely cause of death in scleroderma?
pulmonary!
96
CREST scleroderma
```
Calcinosis
Raynauds
Esophageal dysmotility
Sclerodactyly
Terlangiectasia
- assoc with anticentromere antibody
```
97
acantholysis
separation of epidermal cells- pemphigus vulgaris
98
What do you see on immunofluorescence of pemphigus vulgaris?
potentially fatal IgG antibodies around epidermal cells in a reticular or netlike pattern
99
Pathophys of pemphigus vulgaris?
- potentially fatal autoimmune skin disorder with IgG antibody against desmoglein 3
- positive nikolsky's sign (separation of skin upon manual stroking)
100
difference between pemphigus vulgarus and bullous pemphigoid
BP: autoimmune disodrer with IgG antibody against hemidesmosomes
- spares oral mucosa
PV: involves oral mucosa, IgG against desmoglein 3
101
what is dermatitis herpetiformis?
deposits of IgA at the tips of dermal papillae
| - assoc with celiac disease
102
What are the bugs and drugs assoc with Erythema multiforme?
Bugs: mycoplasma pneumonia and HSV
Drugs: sulfa drugs, B lactams, phenytoin
103
What is lichen planus? assoc?
- Pruritic, purple, polygonal planar papules and plagues
- sawtooth infiltrate of lymphocytes at the dermal-epidermal junction
- assoc with HepC
104
sawtooth infiltrate of lymphocytes at dermal-epidermal junction
lihen planus
105
tumor marker of melanoma
S100
106
mutation seen in melanoma
BRAF kinase- activating
107
Vemurafenib
a BRAF kinase inhibitor- used in melanoma
108
What happens when antibodies bind to their nicotinic ACh receptors in MG?
-results in receptors internalization and degradation by complement fixation
109
which artery runs with the radial nerve?
deep brachial artery
110
What does the FAS gene do?
it deletes clones of autoreactive T lymphocytes
| - thought to be involved in pathogenesis of SLE
