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
Q

what is abciximab

A

ab vs gp 2b3a

good for coranary issues

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26
Q

What is the very best lab to determine DIC

A

↑↑ 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

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27
Q

This causes a waxing/waning LAD that is painless. Very common in adults

A

Follicular lymphoma t(14:18) –> ↑↑ bcl-2

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28
Q

Common in kids
LAD, big liver/spleen
Bone Pain
fever, bleeding

A

ALL

Neoplasms is pre-b or pre-t lymph

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29
Q

Rapidly enlarging nodal mass
Common to involve the GI tract
Typical B symptoms (fever, night sweats)

A

Diffuse large b cell lymphoma

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30
Q

Older men
big spleen
pancytopenia
Usually NO lad

A

Hairy Cell leukemia
cells have hair like projections
+ TRAP

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31
Q

What deficiency develops quicker, b12 or folate

A

Folate def can occur in weeks

b12 will take years

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32
Q

All low molecular weight heparin has this suffix

A

They end in “parin”

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33
Q

In this disorder, factor 5 cannot be inactivated by protein c

A

factor 5 leiden

Clotting is therefore encouraged

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34
Q

What does it mean to have autosplectomy in sickle cell

A

repeated splenic infarct leads to splenic atrophy and fibrosis

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35
Q

What does the white pulp of the spleen do

A

it is lymph tissue

May enlarge in lymph activation or malignancy

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36
Q

What is wrong in acute intermittent porphyria

A

porphobilinogen deaminase deficency

Usually has GI and neuro symptoms

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37
Q

Why does fetal hemaglobin bind oxygen with a higher affinity

A

fetal hemaglobin does not bind 2,3- BPG, and therefor has a higher affinity for hemaglobin

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38
Q

What kind of important met does prostate cancer have

A

osteoblastic bone lesions from mets

not many bone mets are blastic, this one is and therefor important to know

39
Q

Osteoblastic bone lesions cme from what cancers

A

prostate
small cell lung cancer
hodgkin lymphoma

40
Q

This tumor shows a “starry sky” microscopic appearence

A

Burkitt

41
Q

What does C-Myc do

A

Creates a transcription activator if there is a t(14:18) translocation

42
Q

What is the mantle cell lymphoma translocation

A

t(11:14)–> ↑↑ cyclin D1–> promotes G1 to S phase

43
Q

This is a large glycoprotein that binds intigrin, matrix collagen, and serves as a mediator of cell adhesion and migration

A

Fibronectin

44
Q

Adhesion to cells to the extra cellular matrix involves integrin mediated binding to

A

fibronectin, collagen, and laminin

45
Q

Precursor B cells have these CD markers

A

CD10

CD 19

46
Q

Precurser T cells have these CD markers

A

Cd 1, Cd2, Cd 5

47
Q

A myeloblast cancer ie AML will have this unique cell finding

A

Auer Rods

+ Peroxidase positive granules

48
Q

A lymphoblast cancer ie ALL wiill have this marker

A

+ TdT

PAS Positive

49
Q

What is the start codon and what does it actually need to start

A

AUG
Needs a Kozak Sequence of gccRccAUG
where R= A or G

50
Q

What type of anemia is B thalsemia

A

hypochromic microcytic due to decreased beta chain

Unpaired a chains precipitate and mess up erythropoeisis and cause hemoylisis

51
Q

What turns on EPO

A

anemia induced hypoxia can

52
Q

What is aplastic anemia

A

Stem cell failure affecting all lines–> pancytopenia

53
Q

When does a sickle cell sickle

A

When there is low oxygen such as:

ex:
↑ 23 bpg
Low pH

54
Q

What kind of hemaglobin does an embryo have

A

zeta or epsilon
Can pair together or with alpha
this is made by yolk sac

55
Q

When does fetal hemaglobin start

A

8 weeks, up and running well at 14 weeks

a2 g2 (alpha and gama chain)

Made in the liver and spleen

56
Q

When do you have adult hemaglobin

A

around 6 months

Hb A= a2 b2

57
Q

What is hemoglobin a2

A

a2 d2

makes up about 2-3% hb in adults

b thalassemia major tend to have a lot of this

58
Q

Scopolamine is a

A

anti muscarnic - helps with motion sickness

59
Q

What drugs help with vomiting in chemo

A

D antag
5ht antag
neurokinin 1 antag

60
Q

An anemia that forms ringed RBC that are not healthy

The body has iron but cannot use it

A

Sideroblastic anemia
Forms ringed sideroblasts (iron in mitochondria surrounding nucleases makes a ring)

low protoporphyrin- usually from ALAS defect

61
Q

What drug has sideroblastic anemia asa side effect

A

Isoniazide due to the b6 deficiency it creates, which is nesicary for heme synth

62
Q

What is the spleens red pulp for

A

removal of damaged RBCs

63
Q

What is hemaglobin c

A

glutamate replaced by lysine. similar to sickle cell (HbS)
Has less negative charges than S
Moves slow in gel

64
Q

What can help you determine aplastic anemia vs other pancytopenic disease

A

NO SPLENOMEGALLY in aplastic

65
Q

Why does desmopressin help in vWF disease

A

desmopressin helps release more vWF

66
Q

Dificulty swallowing and koilonchia

A

Iron deficiency anemia due to esphogus web
(Plummer Vinson)

koilonchia= spoon nail

67
Q

Would inherited bleeding order show ↑ PT

A

Usually not, inheriting something bad in this path is rare

68
Q

What does a thrombin time measure

A

rate of conversion of fibrinogen to fibrin

defects here are usually liver disease –> ↓ protein production

69
Q

This is a dangerous situation leading to

↑ phos, K, and uric acid

↓ Ca

in the presecence of chemotherapy

A

Tumor lysis syndrome

Cells are rapidly destroyed and spill there contents

Can lead to arythmia (K) or kidney damage (uric acid)

70
Q

How do you prevent uric acid build up in tumor lysis syndrome

A

Allopurinol (xanthine oxidase inhbitor)

Rasburicase (helps make uric acid soluble)

71
Q

What is the most common reason for a prego to get DIC

A

release of tissue factor into circulation from a damaged placenta

72
Q

How can you tell if a drug inhibits factor Xa

A

the name will be

“xaban”

73
Q

What is the inheritence of G6PD

A

x linked resesive

74
Q

What inheritence pattern is vit d resistant rickets

A

x linked dom

75
Q

What does dysplasia represent

A

a reversable change in epithelial cells

Once they breach the basement membrane it is no longer reversable

76
Q

Aquired Causes of sideroblastic anemia

A

Alcoholism- mitochondrial poison
Lead poisoining- ALAD and ferrochelatase problem
Vit b6 problems- ALAS requires this (often from INH)

77
Q

Labs of sideroblastic anemia

A

Iron overload* –> free radicles–> damages cell
↑ ferretin, ↑ serum Iron

Looks similar to hemochromatosis

78
Q

What is thalesmia

A

decreased SYNTHESIS of globin chain

79
Q

thalesmia is protective for

A

P. Falcipurom maleria

80
Q

Types of thalemsemia

A

B and A chain problems

A chain is most important because all blood types use it

81
Q

a thalsemia genetics

A

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
Q

a thalsemia types of knock out

A

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
Q

a thalassemia knock outs. Which is worse?

A

CIS IS WORSE b/c of passing on empty alpha gene

CIS Seen higher in asai

TRANS seen in africa

84
Q

What is Hb H

A

Beta chain tetromer

This s bad, it hurts the RBC

85
Q

What is Hb Barts

A

Gama tetramer in fetus from 4 knockout of alpha gene

Lethal –> hydrops fetalisis

86
Q

How many beta gene for hema-globin

A

2 on chromo 11

11—b—
11—-b—

Disease is gene mutation

complete knock out- Beta 0
diminished B = B+

87
Q

B/ B+ beta thalassemia

A

Beta thal minor

usually asympt- microcytic

key finding isolated increase in hemoglobin A2

88
Q

What is a target cell

A

a bleb of membrane in middle of RBC allowing for hemoglobin

Seen in ↓ cytoplasm (like deflating basketball) –> ↑ membrane

89
Q

B0/ B0 beta thalassemia

A

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
Q

most severe beta thal disease- no beta chains –> a chains tetramerize–> damages RBC

A

beta thalassemia major

the damaged RBC–> ineffective erythropoiesis and extravascular hemolysis

91
Q

Parvovirus B19 infects what

A

erythropoiesis cells

92
Q

What skull x ray finding does beta thal major have

A

crew cut

From extra hematopoiesis in skull–> chipmunk face

93
Q

Purine (thiol) analogs
↓ de novo purine synthesis.
Activated by HGPRT

A

Azathioprine, 6-mercaptopurine

Preventing organ rejection, rheumatoid arthritis, IBD, SLE