Heme/onc Flashcards Preview

UWorld journal > Heme/onc > Flashcards

Flashcards in Heme/onc Deck (93):
1

This process elimatates T Cells than bind to self

Negative selection

2

Where does negative selection of T cells occurs

Thymic medula

3

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

Etoposide

Topo= topoisomerase

Side= sui SIDE al

4

What is generally the purpose of haptoglobin

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

5

What is the CD marker for monocyte- macrophapge lineage

CD 14

6

What is an acanthocyte

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

Typical of abetalipoproteinemia

7

What is a bite cell

Seen in oxidative injury (like g6pd)

Happens when spleen bites out a heinz body

8

What is a target cell

Has bulls eye apperence

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

9

What is a tear drop cell

Seen in myelofibrils

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

10

What is, and how do you treat heprein induced thrombocytopenia

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

Treatment: Direct Thrombin Inhibitor
ex Argatroban

11

What happens to an RBC as it sits near the alveoli

O2 enters the RBC

H+ and CO2 leaves the RBC

This is called the Halden Effect

12

What is the halden effect "equation "

↑ Po2 --> ↑ CO2 and H unloading

In rbcs

13

Folate deficiency inhibits the formation of _______ which limits DNA synthesis

Deoxythymidine monophosphate

Therefor thymadine suplementaion may also help in these cases

14

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

Homocysteine will be elevated in folate deficiency

15

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

Hydroxyurea

16

How can you puff up a sickel cell to avoid polymerazation

Gardos Channel Blocks

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

17

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?

Vit K is cofactor for gamma-glutamyl carboxylase

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

18

Where is heme synthesized

mitochondria

19

What does the partial pressure of oxygen represent

amount of oxygen disolved in the plasma

20

What is enoxaparin

Low molecular weight heprin --> acts mainly on Xa

Binds antithrombin III

Activate AT III --> binds Xa, prevents thrombin formation

21

What is deficiency & general look of porphyria cutanea tarda

uroporphyinogen decarboxylase deficency

this is the more common porphyreria
shows recurring blister on hands

22

what gives bruises a green color after a few days

heme oxygenase

debrades heme into biliverdin (green in color)

23

what is rituximab

ab vs cd20

can improve some lymphoma

24

what is infliximab

ab vs tnf-a

good for RA

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