Heme/onc Flashcards

1
Q

This process elimatates T Cells than bind to self

A

Negative selection

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2
Q

Where does negative selection of T cells occurs

A

Thymic medula

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3
Q

This drug inhibits topoisonerase 2 sealing, leading to DNA breaks and kling of cancer cells

A

Etoposide

Topo= topoisomerase

Side= sui SIDE al

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4
Q

What is generally the purpose of haptoglobin

A

Binds free hemoglobin (from hemolysis) to prevent renal tubular damage

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5
Q

What is the CD marker for monocyte- macrophapge lineage

A

CD 14

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6
Q

What is an acanthocyte

A

RBC with irregular surface.Extreme form called a spur cell.

Typical of abetalipoproteinemia

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7
Q

What is a bite cell

A

Seen in oxidative injury (like g6pd)

Happens when spleen bites out a heinz body

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8
Q

What is a target cell

A

Has bulls eye apperence

Seen in lots of disease: liver obstruction, asplenia, iron def, just… lots…

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9
Q

What is a tear drop cell

A

Seen in myelofibrils

When marrow is replaced by fiber, rbc needs to squeez out. Looks like tear drop

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10
Q

What is, and how do you treat heprein induced thrombocytopenia

A

HIT is when you begin to use up plts and clot after recieving heprin

Treatment: Direct Thrombin Inhibitor
ex Argatroban

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11
Q

What happens to an RBC as it sits near the alveoli

A

O2 enters the RBC

H+ and CO2 leaves the RBC

This is called the Halden Effect

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12
Q

What is the halden effect “equation “

A

↑ Po2 –> ↑ CO2 and H unloading

In rbcs

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13
Q

Folate deficiency inhibits the formation of _______ which limits DNA synthesis

A

Deoxythymidine monophosphate

Therefor thymadine suplementaion may also help in these cases

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14
Q

In a folate deficiency, what are the levels of homocysteine like?

A

Homocysteine will be elevated in folate deficiency

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15
Q

This drug is good in sickel cell, as it increases the synthesis of HbF (which is not messed up)
Reserved for frequent pain crisis

A

Hydroxyurea

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16
Q

How can you puff up a sickel cell to avoid polymerazation

A

Gardos Channel Blocks

These hinder efflex of K and H2O from sickled cell, preventing the dehydration

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17
Q

Alright, so you know that factor 2, 7, 9, and 10 are made in the liver and need Vit K

SO… What chemical rxn is going on with that?

A

Vit K is cofactor for gamma-glutamyl carboxylase

This enzyme Carboxylates factors 2, 7, 9, and 10

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18
Q

Where is heme synthesized

A

mitochondria

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19
Q

What does the partial pressure of oxygen represent

A

amount of oxygen disolved in the plasma

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20
Q

What is enoxaparin

A

Low molecular weight heprin –> acts mainly on Xa

Binds antithrombin III

Activate AT III –> binds Xa, prevents thrombin formation

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21
Q

What is deficiency & general look of porphyria cutanea tarda

A

uroporphyinogen decarboxylase deficency

this is the more common porphyreria
shows recurring blister on hands

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22
Q

what gives bruises a green color after a few days

A

heme oxygenase

debrades heme into biliverdin (green in color)

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23
Q

what is rituximab

A

ab vs cd20

can improve some lymphoma

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24
Q

what is infliximab

A

ab vs tnf-a

good for RA

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25
what is abciximab
ab vs gp 2b3a good for coranary issues
26
What is the very best lab to determine DIC
↑↑ D-Dimer (it is fibrin split product, whcih is the very last step in coag cascade) fibrinolysis problem will mimic DIC but has normal d dimer
27
This causes a waxing/waning LAD that is painless. Very common in adults
Follicular lymphoma t(14:18) --> ↑↑ bcl-2
28
Common in kids LAD, big liver/spleen Bone Pain fever, bleeding
ALL Neoplasms is pre-b or pre-t lymph
29
Rapidly enlarging nodal mass Common to involve the GI tract Typical B symptoms (fever, night sweats)
Diffuse large b cell lymphoma
30
Older men big spleen pancytopenia Usually NO lad
Hairy Cell leukemia cells have hair like projections + TRAP
31
What deficiency develops quicker, b12 or folate
Folate def can occur in weeks b12 will take years
32
All low molecular weight heparin has this suffix
They end in "parin"
33
In this disorder, factor 5 cannot be inactivated by protein c
factor 5 leiden Clotting is therefore encouraged
34
What does it mean to have autosplectomy in sickle cell
repeated splenic infarct leads to splenic atrophy and fibrosis
35
What does the white pulp of the spleen do
it is lymph tissue | May enlarge in lymph activation or malignancy
36
What is wrong in acute intermittent porphyria
porphobilinogen deaminase deficency Usually has GI and neuro symptoms
37
Why does fetal hemaglobin bind oxygen with a higher affinity
fetal hemaglobin does not bind 2,3- BPG, and therefor has a higher affinity for hemaglobin
38
What kind of important met does prostate cancer have
osteoblastic bone lesions from mets | not many bone mets are blastic, this one is and therefor important to know
39
Osteoblastic bone lesions cme from what cancers
prostate small cell lung cancer hodgkin lymphoma
40
This tumor shows a "starry sky" microscopic appearence
Burkitt
41
What does C-Myc do
Creates a transcription activator if there is a t(14:18) translocation
42
What is the mantle cell lymphoma translocation
t(11:14)--> ↑↑ cyclin D1--> promotes G1 to S phase
43
This is a large glycoprotein that binds intigrin, matrix collagen, and serves as a mediator of cell adhesion and migration
Fibronectin
44
Adhesion to cells to the extra cellular matrix involves integrin mediated binding to
fibronectin, collagen, and laminin
45
Precursor B cells have these CD markers
CD10 | CD 19
46
Precurser T cells have these CD markers
Cd 1, Cd2, Cd 5
47
A myeloblast cancer ie AML will have this unique cell finding
Auer Rods | + Peroxidase positive granules
48
A lymphoblast cancer ie ALL wiill have this marker
+ TdT | PAS Positive
49
What is the start codon and what does it actually need to start
AUG Needs a Kozak Sequence of gccRccAUG where R= A or G
50
What type of anemia is B thalsemia
hypochromic microcytic due to decreased beta chain Unpaired a chains precipitate and mess up erythropoeisis and cause hemoylisis
51
What turns on EPO
anemia induced hypoxia can
52
What is aplastic anemia
Stem cell failure affecting all lines--> pancytopenia
53
When does a sickle cell sickle
When there is low oxygen such as: ex: ↑ 23 bpg Low pH
54
What kind of hemaglobin does an embryo have
zeta or epsilon Can pair together or with alpha this is made by yolk sac
55
When does fetal hemaglobin start
8 weeks, up and running well at 14 weeks a2 g2 (alpha and gama chain) Made in the liver and spleen
56
When do you have adult hemaglobin
around 6 months Hb A= a2 b2
57
What is hemoglobin a2
a2 d2 makes up about 2-3% hb in adults b thalassemia major tend to have a lot of this
58
Scopolamine is a
anti muscarnic - helps with motion sickness
59
What drugs help with vomiting in chemo
D antag 5ht antag neurokinin 1 antag
60
An anemia that forms ringed RBC that are not healthy | The body has iron but cannot use it
Sideroblastic anemia Forms ringed sideroblasts (iron in mitochondria surrounding nucleases makes a ring) low protoporphyrin- usually from ALAS defect
61
What drug has sideroblastic anemia asa side effect
Isoniazide due to the b6 deficiency it creates, which is nesicary for heme synth
62
What is the spleens red pulp for
removal of damaged RBCs
63
What is hemaglobin c
glutamate replaced by lysine. similar to sickle cell (HbS) Has less negative charges than S Moves slow in gel
64
What can help you determine aplastic anemia vs other pancytopenic disease
NO SPLENOMEGALLY in aplastic
65
Why does desmopressin help in vWF disease
desmopressin helps release more vWF
66
Dificulty swallowing and koilonchia
Iron deficiency anemia due to esphogus web (Plummer Vinson) koilonchia= spoon nail
67
Would inherited bleeding order show ↑ PT
Usually not, inheriting something bad in this path is rare
68
What does a thrombin time measure
rate of conversion of fibrinogen to fibrin defects here are usually liver disease --> ↓ protein production
69
This is a dangerous situation leading to ↑ phos, K, and uric acid ↓ Ca in the presecence of chemotherapy
Tumor lysis syndrome Cells are rapidly destroyed and spill there contents Can lead to arythmia (K) or kidney damage (uric acid)
70
How do you prevent uric acid build up in tumor lysis syndrome
Allopurinol (xanthine oxidase inhbitor) Rasburicase (helps make uric acid soluble)
71
What is the most common reason for a prego to get DIC
release of tissue factor into circulation from a damaged placenta
72
How can you tell if a drug inhibits factor Xa
the name will be "xaban"
73
What is the inheritence of G6PD
x linked resesive
74
What inheritence pattern is vit d resistant rickets
x linked dom
75
What does dysplasia represent
a reversable change in epithelial cells Once they breach the basement membrane it is no longer reversable
76
Aquired Causes of sideroblastic anemia
Alcoholism- mitochondrial poison Lead poisoining- ALAD and ferrochelatase problem Vit b6 problems- ALAS requires this (often from INH)
77
Labs of sideroblastic anemia
Iron overload* --> free radicles--> damages cell ↑ ferretin, ↑ serum Iron Looks similar to hemochromatosis
78
What is thalesmia
decreased SYNTHESIS of globin chain
79
thalesmia is protective for
P. Falcipurom maleria
80
Types of thalemsemia
B and A chain problems A chain is most important because all blood types use it
81
a thalsemia genetics
Normally 4 alpha alleles on chromo 16 to avoid problems: ``` 16= ---a----a----- 16= ---a-----a---- ``` a thalsemia is a gene deltion 1 knock out= asymptomtic 2 knock out= mild anemia, ↑ RBC
82
a thalsemia types of knock out
1 knock out= asymptomtic 2 knock out= mild anemia, ↑ RBC. Could be trans or cis 3 knock out= HbF protects in utero. --> B2B2 HbH 4 knock out= seen in fetus because termere of gama will kill you (hydrops fetalitis)
83
a thalassemia knock outs. Which is worse?
CIS IS WORSE b/c of passing on empty alpha gene CIS Seen higher in asai TRANS seen in africa
84
What is Hb H
Beta chain tetromer This s bad, it hurts the RBC
85
What is Hb Barts
Gama tetramer in fetus from 4 knockout of alpha gene Lethal --> hydrops fetalisis
86
How many beta gene for hema-globin
2 on chromo 11 11---b--- 11----b--- Disease is gene mutation complete knock out- Beta 0 diminished B = B+
87
B/ B+ beta thalassemia
Beta thal minor usually asympt- microcytic key finding isolated increase in hemoglobin A2
88
What is a target cell
a bleb of membrane in middle of RBC allowing for hemoglobin Seen in ↓ cytoplasm (like deflating basketball) --> ↑ membrane
89
B0/ B0 beta thalassemia
most sever beta thal disease- no beta chains --> a chains tetramerize--> damages RBC Fetus will be fine (HbF does not use beta) s/s: severe anemia by 6 mo, massive erythroid hyperplasia (↑ EPO--> hematopoiesis expansion into skull and stuff)
90
most severe beta thal disease- no beta chains --> a chains tetramerize--> damages RBC
beta thalassemia major the damaged RBC--> ineffective erythropoiesis and extravascular hemolysis
91
Parvovirus B19 infects what
erythropoiesis cells
92
What skull x ray finding does beta thal major have
crew cut From extra hematopoiesis in skull--> chipmunk face
93
Purine (thiol) analogs ↓ de novo purine synthesis. Activated by HGPRT
Azathioprine, 6-mercaptopurine Preventing organ rejection, rheumatoid arthritis, IBD, SLE