Heme/onc Flashcards
This process elimatates T Cells than bind to self
Negative selection
Where does negative selection of T cells occurs
Thymic medula
This drug inhibits topoisonerase 2 sealing, leading to DNA breaks and kling of cancer cells
Etoposide
Topo= topoisomerase
Side= sui SIDE al
What is generally the purpose of haptoglobin
Binds free hemoglobin (from hemolysis) to prevent renal tubular damage
What is the CD marker for monocyte- macrophapge lineage
CD 14
What is an acanthocyte
RBC with irregular surface.Extreme form called a spur cell.
Typical of abetalipoproteinemia
What is a bite cell
Seen in oxidative injury (like g6pd)
Happens when spleen bites out a heinz body
What is a target cell
Has bulls eye apperence
Seen in lots of disease: liver obstruction, asplenia, iron def, just… lots…
What is a tear drop cell
Seen in myelofibrils
When marrow is replaced by fiber, rbc needs to squeez out. Looks like tear drop
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
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
What is the halden effect “equation “
↑ Po2 –> ↑ CO2 and H unloading
In rbcs
Folate deficiency inhibits the formation of _______ which limits DNA synthesis
Deoxythymidine monophosphate
Therefor thymadine suplementaion may also help in these cases
In a folate deficiency, what are the levels of homocysteine like?
Homocysteine will be elevated in folate deficiency
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
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
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
Where is heme synthesized
mitochondria
What does the partial pressure of oxygen represent
amount of oxygen disolved in the plasma
What is enoxaparin
Low molecular weight heprin –> acts mainly on Xa
Binds antithrombin III
Activate AT III –> binds Xa, prevents thrombin formation
What is deficiency & general look of porphyria cutanea tarda
uroporphyinogen decarboxylase deficency
this is the more common porphyreria
shows recurring blister on hands
what gives bruises a green color after a few days
heme oxygenase
debrades heme into biliverdin (green in color)
what is rituximab
ab vs cd20
can improve some lymphoma
what is infliximab
ab vs tnf-a
good for RA
what is abciximab
ab vs gp 2b3a
good for coranary issues
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
This causes a waxing/waning LAD that is painless. Very common in adults
Follicular lymphoma t(14:18) –> ↑↑ bcl-2
Common in kids
LAD, big liver/spleen
Bone Pain
fever, bleeding
ALL
Neoplasms is pre-b or pre-t lymph
Rapidly enlarging nodal mass
Common to involve the GI tract
Typical B symptoms (fever, night sweats)
Diffuse large b cell lymphoma
Older men
big spleen
pancytopenia
Usually NO lad
Hairy Cell leukemia
cells have hair like projections
+ TRAP
What deficiency develops quicker, b12 or folate
Folate def can occur in weeks
b12 will take years
All low molecular weight heparin has this suffix
They end in “parin”
In this disorder, factor 5 cannot be inactivated by protein c
factor 5 leiden
Clotting is therefore encouraged
What does it mean to have autosplectomy in sickle cell
repeated splenic infarct leads to splenic atrophy and fibrosis
What does the white pulp of the spleen do
it is lymph tissue
May enlarge in lymph activation or malignancy
What is wrong in acute intermittent porphyria
porphobilinogen deaminase deficency
Usually has GI and neuro symptoms
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
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
Osteoblastic bone lesions cme from what cancers
prostate
small cell lung cancer
hodgkin lymphoma
This tumor shows a “starry sky” microscopic appearence
Burkitt
What does C-Myc do
Creates a transcription activator if there is a t(14:18) translocation
What is the mantle cell lymphoma translocation
t(11:14)–> ↑↑ cyclin D1–> promotes G1 to S phase
This is a large glycoprotein that binds intigrin, matrix collagen, and serves as a mediator of cell adhesion and migration
Fibronectin
Adhesion to cells to the extra cellular matrix involves integrin mediated binding to
fibronectin, collagen, and laminin
Precursor B cells have these CD markers
CD10
CD 19
Precurser T cells have these CD markers
Cd 1, Cd2, Cd 5
A myeloblast cancer ie AML will have this unique cell finding
Auer Rods
+ Peroxidase positive granules
A lymphoblast cancer ie ALL wiill have this marker
+ TdT
PAS Positive
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
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
What turns on EPO
anemia induced hypoxia can
What is aplastic anemia
Stem cell failure affecting all lines–> pancytopenia
When does a sickle cell sickle
When there is low oxygen such as:
ex:
↑ 23 bpg
Low pH
What kind of hemaglobin does an embryo have
zeta or epsilon
Can pair together or with alpha
this is made by yolk sac
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
When do you have adult hemaglobin
around 6 months
Hb A= a2 b2
What is hemoglobin a2
a2 d2
makes up about 2-3% hb in adults
b thalassemia major tend to have a lot of this
Scopolamine is a
anti muscarnic - helps with motion sickness
What drugs help with vomiting in chemo
D antag
5ht antag
neurokinin 1 antag
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
What drug has sideroblastic anemia asa side effect
Isoniazide due to the b6 deficiency it creates, which is nesicary for heme synth
What is the spleens red pulp for
removal of damaged RBCs
What is hemaglobin c
glutamate replaced by lysine. similar to sickle cell (HbS)
Has less negative charges than S
Moves slow in gel
What can help you determine aplastic anemia vs other pancytopenic disease
NO SPLENOMEGALLY in aplastic
Why does desmopressin help in vWF disease
desmopressin helps release more vWF
Dificulty swallowing and koilonchia
Iron deficiency anemia due to esphogus web
(Plummer Vinson)
koilonchia= spoon nail
Would inherited bleeding order show ↑ PT
Usually not, inheriting something bad in this path is rare
What does a thrombin time measure
rate of conversion of fibrinogen to fibrin
defects here are usually liver disease –> ↓ protein production
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)
How do you prevent uric acid build up in tumor lysis syndrome
Allopurinol (xanthine oxidase inhbitor)
Rasburicase (helps make uric acid soluble)
What is the most common reason for a prego to get DIC
release of tissue factor into circulation from a damaged placenta
How can you tell if a drug inhibits factor Xa
the name will be
“xaban”
What is the inheritence of G6PD
x linked resesive
What inheritence pattern is vit d resistant rickets
x linked dom
What does dysplasia represent
a reversable change in epithelial cells
Once they breach the basement membrane it is no longer reversable
Aquired Causes of sideroblastic anemia
Alcoholism- mitochondrial poison
Lead poisoining- ALAD and ferrochelatase problem
Vit b6 problems- ALAS requires this (often from INH)
Labs of sideroblastic anemia
Iron overload* –> free radicles–> damages cell
↑ ferretin, ↑ serum Iron
Looks similar to hemochromatosis
What is thalesmia
decreased SYNTHESIS of globin chain
thalesmia is protective for
P. Falcipurom maleria
Types of thalemsemia
B and A chain problems
A chain is most important because all blood types use it
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
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)
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
What is Hb H
Beta chain tetromer
This s bad, it hurts the RBC
What is Hb Barts
Gama tetramer in fetus from 4 knockout of alpha gene
Lethal –> hydrops fetalisis
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+
B/ B+ beta thalassemia
Beta thal minor
usually asympt- microcytic
key finding isolated increase in hemoglobin A2
What is a target cell
a bleb of membrane in middle of RBC allowing for hemoglobin
Seen in ↓ cytoplasm (like deflating basketball) –> ↑ membrane
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)
most severe beta thal disease- no beta chains –> a chains tetramerize–> damages RBC
beta thalassemia major
the damaged RBC–> ineffective erythropoiesis and extravascular hemolysis
Parvovirus B19 infects what
erythropoiesis cells
What skull x ray finding does beta thal major have
crew cut
From extra hematopoiesis in skull–> chipmunk face
Purine (thiol) analogs
↓ de novo purine synthesis.
Activated by HGPRT
Azathioprine, 6-mercaptopurine
Preventing organ rejection, rheumatoid arthritis, IBD, SLE
