Exam 2, Lecture 1 - Blood Disorders Flashcards
1
Q
define coagulation/hemostasis
A
a control of bleeding
2
Q
why is coagulation a highly regulated process within the body?
A
coagulation is only needed in areas of bleeding, not systemically
3
Q
what cells are involved in the coagulation process?
A
platelets, endothelial cells, coagulation factors
4
Q
what are coagulation factors, and where are they produced?
A
enzymes made by the liver
5
Q
what are the 3 steps in the coagulation process?
A
- primary hemostasis
- secondary hemostasis
- fibrinolysis
6
Q
what are the 3 steps in primary hemostasis (the first 3 steps of the coagulation process)
A
- vasoconstriction/vasospasm
- platelet adhesion
- platelet activation
7
Q
explain the vasoconstriction phase of primary hemostasis
A
damage to blood vessel causes a release of vasoactive paracrines which cause a spasm, and a constriction of the blood vessel
8
Q
vasoactive paracrines are ____ distance messengers, whereas hormones are ____ distance messengers
A
short, long
9
Q
applying pressure/cold substance to a wound is an example of what?
A
vasoconstriction - triggers vascular spasm which controls the bleeding
10
Q
explain the platelet adhesion phase of primary hemostasis
A
platelets enter the area within seconds of injury. Platelets then bind to integrin receptors
11
Q
How are integrin receptors made, and what do they do?
A
integrin receptors are produced from broken down (damaged) collagen
platelets bind to these receptors during the platelet adhesion phase of primary hemostasis
12
Q
what 3 things are needed for the binding of platelets during the platelet adhesion phase of primary hemostasis?
A
- filipodia (projections)
- integrin receptors
- adhesive glycoproteins
13
Q
what are filopodia?
A
platelets with “spikes”/projections that allow them to bind to integrin receptors
14
Q
what is the Von Willebrand Factor?
A
an adhesive glycoprotien that is needed for adhesion so platelets can stick and aggregate
15
Q
platelet activation phase of primary hemostasis-
In becoming activated, platelets begin to release chemicals from granules. What are the 3 chemicals and their functions?
A
serotonin, ADP, and PAF are all vasoconstrictors and act in a positive feedback loop to attract more platelets to the area
more platelets = more granules = even more platelets
16
Q
platelet activation phase of primary hemostasis-
Which chemical is going to activate the phospholipids on the membranes of platelets?
A
PAF
17
Q
platelet activation phase of primary hemostasis-
Which chemical will cause phospholipids to be converted to thromboxane A2? What is thromboxane A2?
A
PAF will cause phospholipids to be converted to thromboxane A2, which is a vasoconstrictor
18
Q
What is PAF?
A
platelet activation factor
19
Q
what medication are patients put on who are high risk for stroke?
A
low dose aspirin
20
Q
what is the function of aspirin?
A
inhibits thromboxane A2, which is a vasoconstrictor
no vasoconstriction = no platelet aggregation = “thins the blood”
21
Q
what are the 3 phases of the secondary phase of hemostasis in the coagulation process?
A
- initiation phase
- amplification
- propagation phase
22
Q
what is the goal of the initiation phase of secondary hemostasis?
A
to create fibrin!!!
23
Q
What are the 7 steps of initiation phase of secondary hemostasis?
Apologies, number 1 is kinda long
Hint: think cascade, what activates what?
A
- as the endothelial/damaged collagen underwent primary hemostasis, it also activated a coagulation factor known as thromboplastin, or tissue factor (TF)
- TF activates factor 7, which forms a big complex called TF-7 complex
- TF-7 complex activates factor 9 (christmas complex), TF-7 complex also activates factor 10
- factor 10 activates factor 5
- all of these factors activate the enzyme prothrombinase
- prothrombinase converts prothrombin (factor 2) to thrombin
- thrombin causes polymerization of fibrinogen into fibrin
24
Q
What are the 2 steps of amplification phase of secondary hemostasis?
A
amplifying the initiation phase!
- thrombin (made during initiation phase) will cause more platelets to come in and activate factor 5, 8 (also known as von willebrand factor- vWF), and 11
- factor 11a converts more 9 to 9a
25
What are the steps of propagation phase of secondary hemostasis?
1. factor 9a and 8a (8= vWF) will form a complex known as tenase
2. tenase is formed on the platelets and the platelets act as a substrate
3. tenase along w/ phospholipids of platelets + Ca+2, activate more factor 10
4. factors 10a, 5a, phospholipids, and Cas+2 make more prothrombinase to convert more prothrombin to thrombin, and then fibrinogen to more fibrin
26
Simplest terms, what is fibrinolysis known for?
breaking down the clot
27
What are the steps of fibrinolysis?
clot has been formed already and blood is coagulated- what now?
1. clot begins to retract
2. retracting brings the 2 edges of blood vessel together to start healing
3. platelets have contractile proteins (actin and myosin) that will become activated and contract (unknown how)
4. all the fibrin strands are attached to platelets, so when platelets contract, it will pull on the fibrin strands (this pulls the ends of blood vessels together as well, ans is called clot retraction)
5. TPA converts plasminogen into plasmin
6. Plasmin breaks down fibrin and the clot
28
why is it important to clear a clot from the body after coagulation occurs and the blood vessels close?
if the clot is not cleared, it can break away causing an embolism/stroke
it can also cause improper blood flow
29
what is TPA?
tissue plasminogen activator
can be a thrombolytic or fibrinolytic
30
what are patients given in hospitals post stroke to break up the clot before it causes more damage (if it was caught early)?
TPA
31
the body does not rely on only the fibrinolytic pathway - it naturally produces _____ incase the fibrinolytic pathway fails to break down a clot
anticoagulants
32
what are 3 natural anticoagulants that are produced in the body?
heparin
antithrombin 3
protein C
33
what is heparin, a natural anticoagulant, produced by?
mast cells and basophils
34
the body uses 3 ways to break down a clot: the fibrinolytic pathway, anticoagulants, and \_\_\_\_\_\_\_.
chemicals released by undamaged endothelial cells to attack platelets as they pass by
they produce prostacyclin and NO and this prevents platelets from adhering
35
what 2 chemicals are released by undamaged endothelial cells to reduce clotting?
prostacyclin and nitric oxide (NO)
36
what 4 tests should you do to monitor clot formation?
1. platelet count
2. bleeding time test
3. prothrombin time (PT)
4. partial thromboplastin time (PTT)
37
what does the bleeding time test asses?
platelet function
38
what does prothrombin time (PT) asses? (hint: 4 things!!)
1. platelet function
2. clot formation time
3. pathways that **are** tissue factor (TF) dependent
4. monitors effects of warfarin (coumadin)
39
why should you complete a PT test if a patient is on warfarin (coumadin)
patients who have clots are given warfarin - need to monitor to ensure they do not develop too much bleeding
40
what does partial thromboplastin time (PTT) asses? (hint: 4 things!)
1. platelet function
2. clot formation
3. pathways that are **non-**tissue factor (TF) dependent
4. effects of heparin
41
what two natural elements (vitamins) are important for coagulation?
calcium and vitamin K
42
why is vitamin K important for coagulation?
liver needs vitamin K to make coagulation factors 2, 7, 9, and 10
43
what are the two major categories of blood disorders?
proliferative and deficiency disorders
44
what is a proliferative disorder?
making too many blood cells
45
what is a deficiency disorder?
not making enough blood cells
46
how large should RBC be?
7-8 micrometers in diameter
47
what does it mean if a RBC is macrocytic?
larger than normal
48
what does it mean if a RBC is microcytic?
smaller than normal
49
what does it mean if a RBC is poikilocytosis?
variations in shape
50
what does it mean if a RBC is anisocytosis?
variations in size
51
is polycythemia a proliferative or deficiency disorder?
proliferative
52
generally, what is polycythemia?
an activation of RBC, producing more RBC
53
what is a normal hematocrit?
40-45%
54
what is the hematocrit of those with polycythemia?
60-80%
55
What happens as a result of polycythemia?
* increase in blood viscosity (THICK)
* increase resistance in blood vessels
* decreased blood flow
* high risk for producing clots
* heart works harder
56
what can occur as a result of polycythemia, and why?
cardiomegaly - with increased workload, the heart needs to work harder to pump thick blood, gradually increasing the size overtime
57
what is the treatment for polycythemia?
phlebotomy
58
what is anemia caused by - generally?
a decrease in RBC and/or hemoglobin
59
in anemia, decreased oxygen carrying capacity of blood causes what?
extreme fatigue
60
in anemia, decreased oxygen to the heart causes what?
angina and tachycardia
61
in anemia, lack of O2 to regenerate epithelial cells causes what?
ulcers
anemia most commonly causes ulcers in mouth first →stomatitis
62
what are the general signs and symptoms of anemia?
* fatigue
* pallor
* dyspnea
* tachycardia
* angina
* SOB
* changes in hair/skin
63
besides general signs and symptoms, what else occurs in pernicious anemia?
* GI abnormalities
* nausea, vomiting, diarrhea
* paresthesia
* ataxia
* glossitis (smooth tongue)
* B-12 dementia
64
besides the general signs and symptoms, what occurs in iron deficiency anemia?
hematochezia and melena
65
what are the 5 types of anemias we learned in class?
1. pernicious
2. iron deficiency
3. aplastic
4. hemolytic
5. thalassemia
66
which is the most common type of anemia?
iron deficiency anemia
67
in iron deficiency anemia, the RBC are ____ \_\_\_\_\_
microcytic hypochromic
68
what does hypochromic mean?
depletion in color (pale/pink orange for RBC)
69
What problems cause iron deficiency anemia?
1. GI bleeds
2. genital/urinary system
1. malignancy/tumor
2. severe hemorrhoids
3. ulcer
3. decreased iron in diet
4. iron malabsorption
5. heavy menstrual bleeding (premenopausal women)
6. fetus taking iron from mother (pregnant woman)
70
what 2 sources does the body get iron from?
as heme/animal products
ionized iron/plant products
71
heme is _______ transported into cells,
while
ionized iron is _______ with ______ into cells
heme is actively transported into cells
while
ionized iron is co-transported with hydrogen on DMT1 into cells
72
What is DMT1?
divalent metal transporter 1
co-transports ionized iron and hydrogen into cells
73
once heme is transported into the cells, what happens?
enzymes act on heme to ionize iron
74
how is ionized iron transported into the blood stream?
by ferroportin transporters
75
why is absorption of iron highly regulated?
excess iron is toxic to body cells
76
how does the body avoid iron overload?
body will destroy ferroportin transporters to diminish transport of iron into the bloodstream
77
how does the body destroy ferroportin transporters?
1. liver will recognize there is enough Fe and produce a peptide hormone known as hepcidin
2. hepcidin binds to ferroportin
3. lysosomes digest ferroportin transporter and the hepcidin
this prevents Fe from entering bloodstream
78
what happens to excess iron in cells that accumulates from the destruction of ferroportin transporters?
short answer: poop
the body “sluffs” epithelial cells from its intestines all the time, so the iron comes out in your shit and your shit will look black so if your shit looks black you probably have too much iron. remember to always check your poop. it can tell you very important things about your health. i hope neither of u have black shit tonight. or ever.
79
how is iron stored?
as hemoglobin and myoglobin
80
what are the signs and symptoms of iron deficiency anemia?
1. fatigue
2. weakness
3. SOB
4. pallor
5. tachycardia
6. If due to GI bleeding: *hematochezia,* frank blood in stool
7. metabolized blood from GI bleed before it's excreted: *melena,* black tarry stool
81
what is hematochezia?
frank blood (red blood) in stools from a GI bleed (blood that is *not* metabolized before its excreted)
82
what is melena?
black, tarry stools from *metabolized* blood from GI bleed before its excreted
83
what are the 2 treatments for iron deficiency anemia?
1. iron supplements
2. determine and treat cause
84
what is pernicious anemia also known as?
megaloblastic or vitamin B12 deficiency anemia
85
where is pernicious anemia common and not common?
common in european and african descent
not common in people of asian descent
86
T or F: pernicious anemia is an autoimmune dz?
True!
87
pernicious anemia, an autoimmune dz, produces antibodies and cytotoxic T cells against the bodies own _______ cells.
parietal cells
88
how long does it take for signs and symptoms to occur in pernicious anemia? why?
symptoms take roughly a year to develop - this is because the liver stores B12
89
where is vitamin B12 stored in the body?
the liver
90
a person with pernicious anemia has ______ RBCs.
macrocytic RBC (larger than normal)
91
what are macrocytic RBC are also known as?
megaloblasts
92
what causes low levels of vitamin B12? (hint: 4 causes)
1. decreased absorption due to lack of intrinsic factor in gastric mucosa
2. disease (crohn's) causing decreased absorption in the terminal ileum
3. increase in bacteria in intestine - bacteria compete with own cells for B12
4. poor diet
93
What type of enzyme is B12? What does it support?
B12 is a cofactor of DNA synthesis
94
Low levels of vitamin B12 causes abnormal _____ \_\_\_\_\_\_.
RBC formation
95
what do parietal cells in the gastric mucosa produce?
HCl (gastric acid) and intrinsic factor
96
what is HCl needed for?
needed to release B12 from food
97
what is intrinsic factor needed for?
needed to bind to vitamin B12 once released from food
98
how is vitamin B12 absorbed in the body?
1. HCl and intrinsic factor released from parietal cells in gastric mucosa
2. HCl releases B12 from food
3. intrinsic factor binds to B12
4. HCl, intrinsic factor, and B12 all absorbed in ileum together
99
in pernicious anemia, what do autoantibodies attack?
they target hydrogen-potassium ATPase and intrinsic factor
100
what happens when hydrogen-potassium ATPase does not function?
hydrogen ions cant move into the lumen, so HCl cannot be made, and B12 cannot be released from food
101
what happens when autoantibodies attack intrinsic factor?
Vitamin B12 cannot be absorbed - intrinsic factor must bind to B12 to be absorbed into the ileum
102
what is achlorhydria?
a lack of HCl
103
What can happen as a result of pernicious anemia? (hint: 6)
1. chronic atrophic gastritis
2. gastric adenocarcinoma
3. hyper-segmented neutrophils
4. decreased platelets
5. decrease in conduction of nerve impulses
6. other forms of anemia
104
what is chronic atrophic gastritis?
shape change of stomach cells causes loss of function
105
in chronic atrophic gastritis, what shape do the cells transition to?
depends on severity/ stage- normally should be stratified columnar and will decrease size/shape on the severity
1. simple columnar
2. simple cuboidal
3. simple squamous
106
Severe pernicious anemia affects WBC and eventually platelets. What does this cause for WBCs?
hyper-segmented neutrophil (multiple lobes)
107
why do patients with pernicious anemia often have a decrease in conduction of nerve impulses?
demyelination of nerves in spinal tracts and peripheral nerves from lack of B12 cause this
108
what causes glossitis?
pernicious anemia!
1. inflammation of tongue
2. papillae disappear = smooth tongue
109
if patients develop ataxia, what is being affected?
the cerebellum and brainstem
110
patients with pernicious anemia often develop dementia. what is being affected?
neurons of the brain
111
T/F if a patient has B12 dementia, putting them on vitamin B12 can decrease symptoms
true
112
what are the 2 treatments for pernicious anemia?
1. B12 supplementation
2. replacement therapy
113
what is replacement therapy for pernicious anemia?
plasmapheresis to “wash" the blood and get rid of circulating antibodies
114
in aplastic anemia, what is the general problem? (hint 4)
1. bone marrow does not function normally
2. there is a decrease in stem cells
3. pancytopenia (decrease in all cell types)
4. bone marrow tissue replaced by adipose
115
what causes aplastic anemia? (hint 6)
1. 50% idiopathic
2. fanconi's anemia (genetic failure)
3. myelodysplastic syndrome (cancer of immature blood cells)
4. radiation therapy
5. hep C
6. Lupus
116
what is fanconi's anemia?
genetic failure of bone marrow
117
what is myelodysplastic syndrome?
cancer of immature blood cells (they never mature)
118
T or F: bone marrow will return to normal once radiation has stopped
true
119
signs and symptoms of aplastic anemia?
recurrent infections/leukopenia
petechiae (pinpoint bleeding)
120
what is leukopenia?
decrease in WBC
121
what are the treatments for aplastic anemia?
1. if from radiation or dz - treat cause
2. if from immune disorder/genetic - bone marrow transplant
122
what is hemolytic anemia?
change in hemoglobin structure causes RBC to lyse before they should
123
what causes hemolytic anemia?
1. genetic disease
2. incompatible blood transfusion
124
what type of anemia is sickle cell anemia?
hemolytic anemia
125
what causes sickle cell anemia?
1 genetic change
glutamate in one beta chain is replaced by valine
126
in sickle cell anemia, hemoglobin ____ is replaced with hemoglobin \_\_\_\_.
hemoglobin A is replaced with hemoglobin S
127
since patients with sickle cell anemia have hemoglobin S, what happens?
cannot transport oxygen as normal
128
in sickle cell anemia, why do patients have an issue with exercise?
1. they drop off oxygen/RBC lose oxygen
2. hemoglobin becomes polymerized/crystalized
3. forms sickle shape and continually sickles RBCs
129
RBC normally live ____ days. Sickled RBC live ____ days. why?
RBC live 120 days
sickled RBC live 20 days
because sickle shape causes fast lysis of RBC
130
fast lysing of sickled RBC causes what? (hint 3 things)
1. hyperbilirubinemia (too much bilirubin being released into blood)
2. gallstones
3. jaundice
131
what is hyperbilirubinemia?
too much biliruben in blood
132
What does a sickle cell cause?
1. fast lysing
2. easy clumping
133
what does clumping from sickle shape cause?
1. blocks off blood vessels
2. tissues become hypoxic
3. severe pain
4. ischemia (tissues may die)
134
what organs are vulnerable w/ sickle cell anemia?
liver, lungs, heart, kidneys
135
signs and symptoms of sickle cell anemia (in children)
1. splenomegaly
2. dactylitis (hand-foot syndrome)
3. delayed growth
136
why can splenomegaly occur in patients with sickle cell anemia?
1. blood vessels are blocked off
2. lack of blood flow
3. causes tissue death/swelling of spleen
137
children with splenomegaly often deal with what as adults?
their spleen is small and they cannot filter blood
138
what is dactylitis/hand-foot syndrome?
clogged blood vessels in fingers and toes. very painful
139
what is the treatment for sickle cell anemia?
no cure
hydroxyurea
reduce strenuous exercise (especially in children)
140
how does hydroxyurea treat sickle cell anemia?
1. hydroxyurea converts hemoglobin S to hemoglobin F
2. hemoglobin F does not crystalize with low oxygen levels
3. hydroxyurea is metabolized to nitric oxide - a vasodilator
141
who is sickle cell anemia common for?
african descent - sickle cells help to fight off malaria
142
thalassemia is a _____ group of \_\_\_\_\_
thalassemia is a **genetic** group of **anemias**
143
what are the 2 main types of thalassemia?
1. alpha thalassemia
2. beta thalassemia
144
what is alpha thalassemia?
lacking one or both alpha chains of hemoglobin
145
what is beta thalassemia?
lacking one or both beta chains
146
which type of thalassemia is most common?
beta thalassemia
147
beta thalassemia is autosomal \_\_\_\_\_\_
autosomal dominant
148
which patients is beta thalassemia commonly seen in?
those of mediterranean descent
149
being homozygous for beta thalasemia means what for the patient?
that person has thalassemia **major** or Cooley's anemia
150
what is thalassemia major/Cooley's anemia?
* homozygous for beta thalassemia
* lack of all beta chains
* fatal in childhood
151
if a person is heterozygous for beta thalassemia, what does this mean for them?
this person has thalassemia **minor**
152
what is life like for someone with thalassemia minor?
* milder symptoms
* fairly normal life with blood transfusions
* live normal lifespan if mild form of thalassemia minor, more severe form pt usually live to 30s
153
what are signs and symptoms of thalassemia?
1. usual anemia symptoms
2. hemolysis (like sickle cell anemia!)
3. highly reduced oxygen carrying capacity (severe hypoxia)
4. impairs normal growth of children
5. heart failure developed quickly
6. high levels of EPO
154
why do those with thalassemia have a highly reduced oxygen carrying capacity?
* with thalassemia major, they lack 2 beta chains
* lacking heme molecules
* RBC can only carry 2 oxygen molecules
155
why do patients with thalassemia have high levels of EPO?
the kidney realizes they need more RBC (EPO is needed for development of RBC)
156
do high levels of EPO that are naturally produced in a person with thalassemia help increase their RBC #s?
no - it is a genetic issue. the increased EPO will not help genetically they will keep having lower RBC #s
157
what is the treatment for thalassemia?
blood transfusions
158
what is a concern for a patient after multiple blood transfusions? how do you fix this?
* may go into iron overload
* give chelating agents to bind to iron to remove iron from body
159
name 2 white blood cell cancer types
leukemia and lymphoma
160
what is leukemia defined as?
“neoplasms derived from hematopoietic precursors” - especially white blood cells
161
what is the survival rate of leukemia?
45%
162
what is the suffix for an immature cell?
‘blast’
163
what is a mature cell called
cyte
164
what happens to cells in leukemia?
many immature cells multiply out of control in bone marrow and travel to the blood stream
165
what happens to increased immature WBCs in leukemia?
no immune protection! the immature WBC cannot protect you
166
what are the 2 main groups of leukemia?
acute and chronic leukemia
167
what are two types of acute leukemia?
1. acute lymphatic leukemia (ALL)
2. acute myelogenous leukemia (AML)
168
does ALL occur/progress rapidly or slowly?
rapidly - by the time you realize you're sick its usually too late
169
what is the most common childhood leukemia?
80% of all childhood cancers are ALL
170
In ALL, patients have an increased number of \_\_\_\_\_\_
lymphoblasts - abnormal B and T cells precursors
171
what is ALL genetically associated with?
translocation
172
what is the general issue in AML?
1 or more granulocyte cell derived from myeloid stem cells
173
what 3 cells are affected in AML?
basophils
eosinophils
neutrophils
174
AML occurs in ______ (adults or children)
adults
175
T or F: there is 1 type of AML
FALSE - there are many subtypes of AML
(we do not need to know the specific subtypes)
176
how can you tell a patient has AML from a blood smear?
you will see crystalline inclusions (auer rods/auer bodies) on blood cells
177
what are auer bodies/auer rods?
crystalline inclusions seen on blood cells from a patient with AML
178
what can cause AML? (hint: 3)
1. chemo
2. radiation
3. genetic translocation
179
what are the two types of chronic leukemia?
CLL - chronic lymphocytic leukemia
CML - chronic myelogenous leukemia
180
do chronic leukemias have a rapid or slow onset?
slow onset
181
CLL is a neoplasm of ______ \_\_\_\_ \_\_\_\_\_
CLL is a neoplasm of **mature B cells**
182
T or F: in CLL, T cells are malfunctioned
FALSE - In CLL, B cells are affected, not T cells
183
CML is an indolent form of \_\_\_\_
AML
184
which chromosome is translocated in CML?
Philadelphia chromosome = translocation of chromosomes 9 and 22
185
what are 3 possible outcomes of leukemia?
1. anemia
2. thrombocytopenia
3. bone pain
186
why do patients with leukemia often develop anemia?
1. bone marrow produces immature blast cells or granulocytic cells
2. takes up space in bone marrow
3. precursors of RBC are decreased in number, as well as platelets
187
why do patients with leukemia often develop thrombocytopenia?
1. overproduction of immature cells takes up space in bone marrow
2. precursors of platelets decrease due to less room
188
what is a normal platelet count?
150,000
189
what do platelet numbers decrease to in a patient with thrombocytopenia?
10,000
190
why do patients with leukemia often have bone pain?
1. cells build up and push/impinge on nerve
2. stretch periosteum around bone
3. bone pain
191
what are the 6 treatment options for those with leukemia?
1. chemo
2. interferon
3. proper nutrition
4. alkalinize urine of patients
5. blood transfusions
6. bone marrow transplant
192
why does chemo increase the changes of kidney stones and gout?
1. chemo kills cells
2. destroy nucleic acid
3. nucleic acids into blood stream
4. increases uric acid
5. causes hyperuricemia
6. kidney stone and gout
193
what are 2 risks associated with chemo treatment?
kidney stones and gout
194
why is proper nutrition important for those with leukemia?
important due to lack of WBC
195
why would you need to alkalinize patients urine if they have leukemia?
if they have hyperuricemia - to prevent kidney stones
196
why would a person with leukemia need a blood transfusion?
if granulocytes, thrombocytes, or platelets numbers are low
197
what are the signs and symptoms of leukemia? (hint: 6)
1. infections
2. hemorrhage
3. enlarged spleen, liver, etc.
4. headaches and blurred vision
5. bone pain
6. fatigue
198
why might a patient with leukemia have headaches and blurred vision?
if cells infiltrate blood vessels of the CNS
199
how do you test for leukemia?
1. blood test
2. bone marrow biopsy *always* to confirm
200
what drugs can induce thrombocytopenia?
1. aspirin
2. digoxin
3. furosemide
4. vancomycin
etc.
201
what are the signs and symptoms of drug induced thrombocytopenia?
petechiae on skin or mucus membranes
202
T or F: in drug induced thrombocytopenia, signs and symptoms will subside 1 week after drug is discontinued.
FALSE - symptoms will subside in **2-3 days**
203
how do quinine and NSAIDs induce thrombocytopenia?
they tigger antibodies that bind to platelets and destroy them
204
how does heparin induce thrombocytopenia?
1. binds to PF4 (factor released from alpha granules)
2. triggers immune response with IgG
205
what are 3 blood clotting disorders we learned?
hemophilia
von willebrand disease
DIC
206
blood clotting disorders cause _____ or _____ bleeding
**excess** or **spontaneous** bleeding
207
what are possible causes of blood clotting disorders?
1. Thrombocytopenia
2. Coumadin/heparin (drugs)- anticoagulants
3. Chemo
4. Radiation
5. Cancers
6. Hemorrhagic fever viruses – Ebola
7. Aspirin and NSAIDs
8. Vitamin K deficiency/liver disease
208
why might a person with vitamin K deficiency or liver disease develop a blood clotting disorder?
liver needs vitamin k to make coagulation factors
209
what are signs and symptoms of blood clotting disorders?
1. Frequent nose bleeds and bleeding gums
2. Decrease BP – hypotensive
3. Tachycardia – heart compensates and pumps harder/faster
4. Petechiae
5. Blood in feces
6. Ecchymosis (large bruises)
7. Hematemesis – vomiting blood that looks like coffee granules
8. Hemarthrosis – bleeding into joints
9. Hemoptysis – coughing up blood
210
is hemophilia A x-linked recessive or x linked dominant?
x linked recessive
211
what are signs/symptoms of hemophilia?
1. hematomas
2. hemarthrosis
3. hematuria
4. blood in stool
212
why will a patient with hemophilia develop hematomas?
they have constant slight bleeding
213
what is hemarthrosis? who is it common in?
1. bleeding into the joints
2. red, hot, swollen, painful
3. common in those with hemophilia
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what is hematuria?
blood in urine
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what are 4 tests that are used to diagnose hemophilia?
1. bleeding time
2. PT
3. PTT
4. coagulation time
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in a patient with hemophilia what tests will be normal?
**bleeding time** test and **PT** will be normal
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what tests will be abnormal for those with hemophilia?
**PTT** and **coagulation time** are prolonged
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what are the 4 treatments for hemophilia?
1. prevent children from too much activity
2. DDAVP- desmopressin
3. ADVATE (recombinant factor 8)
4. NPLATE
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what is desmopressin and what does it treat?
increases clotting factors by causing endothelial cells to release factor 8 (vWF)
treats hemophilia
220
what is ADVATE?
recombinant/genetically made factor 8 to treat hemophilia
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what is NPLATE?
increases platelet production in bone marrow in hemophilia
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what are 3 types of hemophilia?
hemophilia **A, B,** and **C.**
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hemophilia A is a lack or deficiency of _____ \_\_.
**factor 8**
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in hemophilia A, women are \_\_\_\_\_, while men _____ the disease
women are **carriers,** men **manifest** the disease
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which is the most common type of hemophilia?
Hemophilia **A** - 1 in every 5,000 men have the disease
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what is hemophilia B also known as?
“christmas disease”
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are hemophilia B and C common or rare?
RARE
228
what is hemophilia B a deficiency of?
factor 9 (christmas factor)
229
what is hemophilia C also known as?
rosenthal syndrome
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what is hemophilia C a deficiency of?
factor 11
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von willebrand disease is a deficiency of what?
vWF (factor 8)
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what does a deficiency of vWF cause?
1. no factor 8 = **no clotting**
2. factor 8 binds to vWF to help platelets stick together/to blood vessel walls
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what is the most common hereditary clotting disease?
Von Willebrand disease
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is Von willebrand disease autosomal dominant or resessive?
autosomal dominant
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there are 3 types of von willebrand disease. how do they vary?
they vary based on **severity**
236
what are the signs and symptoms of von willebrand disease?
1. hematomas, hemarthrosis, hematuria (like hemophilia)
2. frequent nose bleeds
3. easy bruising
4. women have abnormally heavy menstrual bleeding
similar to hemophilia S&S, but milder
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how do you treat mild von willebrand disease?
patients are only treated if they need to undergo a procedure
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how do you treat severe form of von willebrand disease?
desmopressin
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generally what is DIC?
severe/excess blood clotting **and** excess bleeding
240
what does DIC stand for?
**disseminated intravascular coagulation**
241
how does excessive bleeding occur in those with DIC?
1. systemic clotting in all blood vessels causes tissues to die
2. liver cannot produce coagulation factors quick enough
3. use up all platelets and clotting factors
4. results in excessive bleeding **after** excessive clotting
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True or false: In DIC, excessive bleeding occurs before excessive clotting
FALSE - excessive clotting occurs first, then excessive bleeding
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what are 4 causes of DIC?
1. usually from complication of a medical problem
2. after trauma
3. post infection
4. complication of childbirth
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what are signs and symptoms of DIC?
1. blood pressure decreases
2. can go into shock
3. thrombocytopenia
4. petechiae
5. stroke and seizures
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why might a person with DIC have stroke or seizures?
if their clots move into CNS
246
what is treatment based on for DIC?
depends on which phase they're in - coagulation or bleeding phase
247
how do you treat DIC?
find trigger and treat the cause
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Coagulation factors are constantly circulating in bloodstream. What form are they in- active or inactive?
they are in inactive form until they are needed to be activated by the cascade coagulation system
249
What does it mean if a coagulation factor has an “a” attached to it?
that coagulation factor has been activated
250
Once platelets bind to the integrin receptors and all the cells are clogging together, what does this mean during primary hemostasis?
they are activated
251
What causes a platelet plug during primary hemostasis? What is purpose of plug?
all the platelet adhesion causes a platelet plug to the damaged area
this will prevent any more blood loss from damaged vessel
252
step 1 of initiation phase of secondary hemostasis-
As the endothelial/damaged collagen underwent primary hemostasis, it also activated a coagulation factor that is important for this next process. What is this coagulation factor called?
thromboplastin, or tissue factor (TF)
253
step 2 of initiation phase of secondary hemostasis-
TF activates what factor? What does this cause?
TF activates factor 7, which forms a big complex called TF-7 complex
254
step 3 of initiation phase of secondary hemostasis-
TF-7 complex activates what two factors?
factor 9 and 10
factor 9 is also known as the christmas factor
255
step 4 of initiation phase of secondary hemostasis-
Factor 10 activates what?
factor 5
256
step 5 of initiation phase of secondary hemostasis-
What factors activate the enzyme prothrombinase?
all of them!!!!
257
step 6 of initiation phase of secondary hemostasis-
prothombinase converts what to what?
prothrombin (factor 2) to thrombin
258
step 7 of initiation phase of secondary hemostasis-
thrombin does what?
causes polymerization of fibrinogen into fibrin, which is an insoluble strands of protein
259
Fibrin is needed for what during the amplification phase of secondary hemostasis?
to start wrapping around platelets to make clots in the damaged area and control bleeding
260
step 1 of amplification phase of secondary hemostasis-
thrombin causes more platelets to come into area and activate what factors?
factor 5, 8 (also called vWF), and 11
261
step 2 of amplification phase of secondary hemostasis-
factor 11a converts more what?
9 to 9a
262
step 1 of propagation phase of secondary hemostasis-
What two activated factors form a complex known as tenase?
factor 9a and 8a form complex called tenase
263
step 2 of propagation phase of secondary hemostasis-
tenase is being formed where? What acts as substrate?
platelets
264
step 3 of propagation phase of secondary hemostasis-
tenase along with phospholipids of platelets and Ca+2 activate more…?
factor 10
265
step 4 of propagation phase of secondary hemostasis-
factors 10a, 5a, phospholipids, and Ca+2 make more of what? What does this do?
makes more prothrombinase to converts prothrombin to more thrombin
AND
more fibrinogen to more fibrin
266
What are normocytic cells?
normal blood cells (no variation/deviation)
267
What is vitamin B12 also known as?
cobalamin
268
T/F:
At the beginning of pernicious anemia, hemoglobin and hemoglobin synthesis appear normal
true!!! symptoms take a long time to develop
269
What are the S&S of sickle cell anemia for adults & kids (besides the usual)?
1. increase in infections
2. obstruction of blood vessels results in increased peripheral resistance
3. obstruction and infarction results in ulcers
270
How does the cell differ from a normal cell in thalassemia?
* microcytic
* hypochromic
* anisocytosis
271
Generally speaking, what is thalassemia caused by?
genetic defect to Hb
272
What is the hormone needed for erythropoiesis (development of RBCs)?
erythropoietin (EPO)
273
CLL is a cancer most prominent in what age group?
adults 50+
