Hematology/Oncology Flashcards
1
Q
Where does hematopoiesus occur in embryo?
3rd-7th month?
7th month?
at birth?
adulthood?
A
Embryo: Yolk sac
3rd-7th month: spleen and liver
7th month: Marrow cavity
Birth: Mostly bone marrow, liver/spleen as back-up
Adulthood: Bone marrow (Skull, ribs, sternum, vertebral columns, proximal ends of the femur)
2
Q
Hematopoiesis starts with a ________ stem cell.
A
Pluripotent
3
Q
This is a bone marrow stem cell that has the potential to become any blood cell.
A
Hematocytoblast
4
Q
Hematocytoblasts can differentiate into ________ or ________
A
Myeloid stem cells
Lymphoid stem cells
5
Q
A myeloblast will become a _______, ________, or _________.
A Monoblast will become a ____________ or ________
A erythroblast will become a ___________.
A Megakaryoblast will become a ___________.
A
Neutrophil, Eosinophil, Basophil
Monocyte or macrophage
Erythrocyte
Megakaryocyte
6
Q
WBC precursors outnumber RBCs :
A
3:1
7
Q
How long is a WBC’s life span?
A
3 days
8
Q
What is the main growth factor for erythroblasts?
A
Erythropoietin (EPO)
9
Q
Where is EPO formed?
When is it released?
A
Kidney (by peritue)
When pO2 decreases
10
Q
Once a blast is committed to becoming an erythrocyte, ____-____ also stimulates cell division
A
GM-CSF
11
Q
What is the main growth factor for thromboblasts?
A
Thrombopoietin
12
Q
What FOUR “cytokines” work to increase platelet numbers?
Which one is a fever inducer?
A
GM-CSF
IL-3
IL-6 (fever inducer)
IL-11
13
Q
What THREE “cytokines” work as the main growth factors for myeloblasts?
A
G-CSF
GM-CSF (increases monocytes = more macrophages)
IL-3
14
Q
What SIX molecules are important to RBC formation?
A
Iron EPO Heme Hemoglobin Folate B12
15
Q
T/F: Folate and B12 are made by the human body
A
False (they are not)
16
Q
Where is B12 obtained from?
A
Small Intestines
Stomach
17
Q
What most B12 be bound to in order to be absorbed?
Where is this made?
Where does B12 ultimately end up being absorbed?
A
Intrinsic factor
Stomach
Ilium
18
Q
T/F: B12/Folate deficiencies cause anemia because of decreased RBC production, not due to absence of iron
A
True
19
Q
______ anemia is described as larger cells than usual (seen in labs as high MCV)
A
Macrocytic
20
Q
______ anemia is described as more Hb per cell than normal (seen in labs as high MCH)
A
Hyperchromic
21
Q
What is the main component of RBCs?
A
Hemoglobin
22
Q
Typically hemoglobin is made up of 2 _____ and _______ chains.
A
Alpha
Beta
23
Q
What are SIX hemoglobin variants?
A
Four α-Hb genes Two β-Hb genes Four γ-Hb genes Two δ-Hb genes Two ε-Hb genes Two ζ-Hb genes
24
Q
This Hgb variant begins in the third trimester and can have a normal adult range of 1.5 - 3.5%
A
Hemoglobin A2 (alpha 2 delta 2)
25
This Hgb variant is also referred to as fetal hemoglobin and can be elevated in patients with sickle cell disease or beta-thalassemia.
Hemoglobin F (alpha 2 gamma 2)
26
This is referred to a large chemical group called a porphyrin, made of 4 rings of porphobilinogen attached to a central iron ion
Heme
27
What are 4 others molecules that use heme?
1. Myoglobin (o2 binding in muscle)
2. NO synthase (vasodilation)
3. Cytochromes
4. Catalase
28
Where is heme primarily synthesized?
Liver and bone marrow
29
During the process of heme formation, glycine binds to SuccCoA (Kreb's Cycle) to form ________, which join together to form _______. Four of these come together to form __________ and then ___ is added to the middle to form heme.
D-ALA
Porphobilinogen
Porphyrin
Fe2+
30
This is an inherited or acquired disease affecting heme production, has multiple subtypes, and is unique for turning feces purple when it is exposed to light.
Porphyria
31
What are the Sx of porphyria?
```
Muscle Pain
Tooth Changes
Increased Hair growth
Anemia
Sun Intolerance
Neuropathies
Personality changes
```
32
T/F: Iron for heme is not absorbed in the GI tract
False (It is absorbed in the GI tract)
33
Heme is stored as _______ and transported as _______
Ferrin
Transferrin
34
What vitamin increases iron absorption?
What decreases it?
Increases: Vitamin C
Decreases: Ca2+, Mg2+
35
Microcytic hypochromic anemia is often a result of _____ deficiency
Iron
36
What is the lifespan of a RBC?
100-120 days
37
What unique feature of a RBC's plasma membrane allows it to pass through small capillaries?
What allows it to have an easier time during O2 exchange?
Flexibility
High Surface area
38
___ of RBCs hemolyze during circulatory life.
___ of RBCs survive long enough to have their shape altered by wear and tear.
10%
90%
39
______ is the apoptosis of a RBC
Eryptosis
40
Where are the sites of RBC breakdown (FOUR)?
1. Liver
2. Spleen
3. Bone Marrow
4. Lymph Nodes
41
Hemoglobin is recycled by separating heme from ______ and then turning that into amino acids.
The heme is then carried to the ______ by a protein which is brocken down into ________ then into bilirubin to be used in the bile, however some is converted to a brown pigment by _______ and excreted in the feces.
Globin
Liver
Biliverdin
Bacteria
42
_____ anemia is when you do not make enough RBCs mostly do to dysfunctioning bone marrow.
Aplastic
43
________ anemia occurs when there is not enough Hgb.
Microcytic
44
________ ________ anemia occurs when RBCs die too fast.
Autoimmune hemolytic
45
This is the congenital absences of the protein spectrin that give RBC's their shape.
Hereditary Spherocytosis
46
If the Hgb dissociation curve shifts to the left then Hgb is holding onto 02 more ______
tightly
47
If the Hgb dissociation curve shifts to the right then Hgb is holding onto 02 more ______
loosely
48
An increase in DPG levels will cause Hgb to hold onto O2 ________ shifting the dissociation curve to the _____.
loosely
right
49
As temperature decreases, Hgb will hold onto 02 ________ shifting the curve to the ______
tightly
left
50
As pH decreases becoming more acidic, Hgb will hold onto O2 _______ shifting the curve to the ______.
loosely
right
51
Between myoglobin, hemoglobin F, and hemoglobin A, which is the "strongest holder" of 02?
Myoglobin
| fetal hemoglobin is next strongest
52
This disease occurs when there is 2 alpha and beta chains that make up Hgb however the beta chains are abnormal
(Also called Hemoglobin SS)
Sickle Cell Disease
53
This abnormal form of hemoglobin occurs when there are 2 alpha and beta chains that compose Hgb however only ONE beta chain is some hemoglobins are abnormal.
(Also called Hemoglobin AS)
Sickle Cell Trait
54
T/F: HgbS tends to change shape at a higher pH
False (They change shape at a lower pH)
55
_____ thalassemia occurs when one or more of the four alpha globin genes.
Alpha
56
Hemoglobin H is 4 _____ chains bonded together.
beta
57
Hemoglobin Barts is four _____ chains bonded together.
gamma
58
______ thalassemia occurs when one or more of the two beta globin genes are missing.
Beta
59
_________ is Hgb that exists with Fe3+ rather than Fe2+.
Will this form of Hgb hold onto oxygen better or worse than Fe2+ binded Hgb?
Methemoglobin (HbM)
Worse (Fe3+ will essentially not hold 02 at all and will remain in the doxygenated shape)
60
How is methemoglobin corrected?
What Pathway?
Using NADH made it the Embden-Meyerhof Pathway
61
T/F: Usually involves a defect on cytochrome B5 Reductase (methemoglobin reductase)
True
62
What is considered the normal amount of methemoglobin?
When would you become cyanotic?
Death?
<1.5 (10% of hemoglobin)
1.5 - 3.0 (10-20% of hemoglobin)
>10.5 (>70% of hemoglobin)
63
What are three common causes of methemoglobin?
1. Hb variants [rare]
2. Cytochrome B5 Reductase deficiency [rare]
Toxic substances [more common]
64
High exposures to what three oxidizing chemicals increase the risk for methemoglobin?
Anesthetics?
Abx?
1. Nitrates
2. Benzocaine
3. Dapsone
Prilocaine
Trimethoprin and sulfas
65
What is the name of the pathway RBCs use to get fuel?
What molecule do they use as fuel?
Embden-Meyerhof Pathway
Glucose
66
________ _________ ______ is a “detour” that some G6P take instead of going through glycolysis that occurs to generate NADH, a reducing agent that detoxes free radicals
Hexose Monophosphate Shunt
67
This is a deficiency most commonly found in men, is X-linked, and affects infants more than children and children more than adults.
G6PD Deficiency
68
Being heterozygous for G6PD deficiency protects against _____.
Why?
Malaria, because G6PD deficiency destabilizes the RBC membrane making it difficult for the parasite to reproduce.
69
______are fragments of megakaryocytes that leave the bone marrow and move into systemic circulation w/o nuclei or membrane bound organelles, w/ a lot of surface receptors and many granules
Platelets
70
What is the lifespan of a platelet?
What is the lifespan of a Neutrophil?
What is the lifespan of a Basophil?
What is the lifespan of a Eosinophil?
10 days
1-3 days
3-10 days
10 - 21 days
71
Myeloblasts in the peripheral blood is a sign of what?
Leukemia
72
Should Promyelocytes or Myelocytes be found in the peripheral blood?
Should Metamyelocytes, bands, or segmented neutrophils be found in the peripheral blood?
No
Yes
73
An increase in bands in the peripheral blood is indicative of what?
Acute infection
74
______ is a decrease in myeloid cells (RBCs, Neutrophils, and/or platelets)
What are THREE examples of this?
Cytopenia
Leukopenia
Anemia
Thrombocytopenia
75
What are THREE ways cytopenia can occur?
1. Increased destruction
2. Increase sequestration
3. Decreased production
76
______ is a decrease in all THREE myeloid lines.
Pancytopenia
77
_______ pancytopenia occurs when there is increased destruction of myeloid cells, likely autoimmune.
_______ pancytopenia occurs when there is decreased production of myeloid cells, likely due to environmental exposures (chemicals chemo, disease, etc...)
Idiopathic
Secondary
78
Which or what growth factors are decreased in iron deficiency?
Kidney disease?
Liver disease?
Folate/B12
EPO
CSFs
79
___________ syndrome occurs when there are dysfunctional stem cell lines in the bone marrow, is a precursor to acute myeloid leukemia, and can require a bone marrow transplant.
Myeloidysplastic
80
_______ anemia is totally dysfunctional stem cells in the bone marrow.
A biopsy of the bone marrow in this condition would show decreased stem cell replace by ______.
Aplastic
Fat
81
What are the most common causes of aplastic anemia?
| There are Seven
1. Idiopathic (>65%)
2. Chemicals (Benzene)
3. Drugs (Chemo, etc...)
4. Radiation
5. Viruses (Hep C, Paravirus B19, EBV)
6. Inheritied Disease (Fanconi Anemia()
7. Other (Connective tissue disorders, pregnancy)
82
How is aplastic anemia treated?
1. Androgens and CSFs
2. Cyclophosphamide (a chemo agent)
3. Bone Marrow Transplant
83
Idiopathic pancytopenia (also referred to as _________ ______) appears to be an autoimmune destruction of myeloid elements in the peripheral blood by macrophages that were inappropriately amplified by helper T cells. Decrease in all myelocytic cell lines
Hemophagocytic Syndrome
84
What would you expect the bone marrow, spleen, and liver to be filled with in a patient with hemophagocytic syndrome?
macrophages that have phagocytosed RBCs
85
T/F: Hepatosplenomegaly is not common in patients with hemophagocytic syndrome
False (It is common)
86
What organ is the most common site of RBC sequestration in a patient with sickle cell disease?
Spleen
87
What commonly triggers sickling episodes?
Infection
| Hypoxia
88
What are three risks for splenic sequestration?
How would this be treated?
How fast would the spleen die without treatment?
1. Acute/Severe hypovalemia
2. Hypovolemic shock
3. Splenic rupture
Tx:
1. Transfusion
2. BP Support
Splenic death in 2 hours
89
T/F: Sickle cell disease that does not cause blood vessel occlusion
False (It does)
90
This is known as spleen death due to narrow blood vessels in spleen.
(It has often died by the time a sickle cell pt is 2-5 y/o)
Autosplenectomy
91
T/F: Platelets contain nuclei and organelles
False
92
T/F: Platelets contain granules which contain secretory products to aid in clotting
True
93
This is a potent platelet aggregator and vasoconstrictor that is localized to the site of injury and activated platelets
TXA2
94
_____ is produced by endothelial cells around the clot to reverse the effects of TXA2
PGI2
95
What enzyme does ASA inhibit?
COX-1
96
Why can't platelets produce more COX-1 enzymes in a patient taking ASA?
Platelets have no nucleus, and come “pre-loaded” with enzymes they will need, and cannot make more
97
What is the extrinsic pathway in the clotting cascade?
Intrinsic?
Trigger→ 7→ 9→ 10→ 2→ 1
Trigger→ 12→ 11→ 9→ 10→ 2→ 1
98
What factors in the clotting cascade are amplifiers?
stabilizers?
Factor 5, 8
Factor 13
99
Where are clotting factors produced?
Are they secreted in their active or inactive forms?
Liver
Inactive Forms
100
What is used to treat factor disorders?
FFP
101
_________ is a genetic disorder characterized by deficiencies in clotting factors (usually Factor VIII)
Hemophilia
102
PT is used to measure ________ pathway activity and asses vitamin ___ status.
Extrinsic
K
103
aPTT is used to measure the _______ pathway and is useful in checking factor __.
Intrinsic
8
104
A low PT is indicative of a ______ ability to clot
A high PT is indicative of a _____ ability to clot
high
Low
105
T/F: Hemophilia A (Factor VIII deficiency) is a X linked disorder
True
106
By what age are patients typically diagnosed with Hemophilia A?
3 y.o.
107
What is a hallmark of hemophilia A?
Hemarthrosis
108
Less than 50% Factor VIII activity is considered?
6-49% Factor VIII activity is considered?
2-5% Factor VIII activity is considered?
0-1% Factor VIII activity is considered?
Disease
Mild
Moderate
Severe
109
How is hemophilia A treated?
Factor VIII replacement
110
T/F: von Willebrand disease is the most common inherited clotting disorder
False (It is the most common inherited bleeding disorder)
111
von Willebrand disorder involves an autosomal dominant of chromosome ____.
16
112
How is von Willebrand factor improtant in the clotting cascade?
What happens in von Willebrand disorder?
vWF allows Factor VIII to travel through the blood bound to it
Without vWF, Factor VIII has a very short lifespan
113
What are classic presenting Sx in some with vWF deficiency?
What is the classic complaint in women with vWF deficiency?
Epistaxis
Bleeding gums
Heavy menstrual cycle
114
Vitamin ____ is necessary for synthesis of multiple clotting factors.
Lack of this vitamin can present as severe bleeding that is difficult to control
Vitamin K
115
Protein __ and __ require vitamin K to be produced.
Protein C and S
116
What are good sources of vitamin K
Leafy Green Vegetables
Cauliflower
Bacteria in the colon
117
T/F: Fetuses can create and store vitamin K before birth
False (They can not, it travels through the placenta)
118
Why are fetuses given Vitamin K injections at birth?
To protect again hemolytic diseases
119
28-y/o woman admitted due to a tendency to hemorrhage. She used to be obese in childhood but a fear of obesity forced her to go on a diet after she entered high school. For the past few years, she has had episodes of self-induced vomiting after each meal, often in conjunction w/ purgative (ipecac) use. Her menstruation is irregular, occurring only a few times/year. On admission, she is 5’0” and 77 lb. PE reveals numerous ecchymoses of her extremities, right flank and hip and hemarthrosis of her rt. knee.
Labs:
```
Hematocrit 28%
Platelet count was 574 x 109/l
PT = 99.1sec (normal, 10-13 sec)
aPTT = 52.9 sec (normal, < 45)
Coag factors II, VII, IX and X markedly reduced
Fibrinogen normal
```
Why are her lab values abnormal?
How would you treat this?
The body’s Vit K stores tend to be small, thus frequent vomiting/purging episodes can result in deficiency of Vit K & Vit K-dependent coagulation factors
Tx: IV Vit K w/ rapid resolution of her coagulopathy
120
______ & ________ inactivate unbound thrombin in the systemic system
Antithrombin III & protein C
121
______ is an anticoagulant contained in mast cells, basophils, and on surface of endothelial cells which enhances antithrombin III and inhibits the intrinsic pathway
Heparin
122
T/F: Heparin will dissolve clots
False
123
When ______ __ is activated it turns off the amplification activity of Factors V and VIII, which greatly ____ the clot formation process
Protein C
Slows
124
T/F: Protein C cannot function with Protein S
True
125
Would a person with Protein C deficiency be at risk for increased bleeding or increased clotting?
Increased clotting
126
______ is a clot that forms and persists in an unbroken blood vessel, which can block the vessel if large enough, leads to ischemia and tissue death downstream from clot
Thrombus
127
_____ is a clot that is free-floating in bloodstream
Embolus
128
Are inflammation, atherosclerosis, DM, and HTN all risk factors of clotting?
What are TWO "non-disease" risk factors for clotting?
Yes
Bed ridden patient
Long flights
129
What are three drugs commonly used to prevent clotting?
1. ASA
2. Warfarin
3. Heparin
130
TPA converts plasminogen to _____ and is typically used to _____ clots.
Plasmin
Dissolve
131
How do you treat Protein C deficiency?
Anticoagulants (Heparin followed by warfarin)
132
This disorder is a mutation of Factor V in which is has "no shut off valve" for protein C to bind to?
Factor V Leiden Mutation
133
T/F: DVTs, Thrombophlebitis, and PEs less common in patients with Factor V Leiden
False (Those are more common)
134
What are two tests used to diagnose Factor V Lieden?
1. Dilute Russell's Viper Venom Test
| 2. DNA Electrophoresis with restriction endonucleases
135
What composes Virchow's Triad?
| THREE Things
1. Stasis of blood flow
2. Endothelial Injury
3. Hypercoagubalility
