Hemolytic Disorders (Exam 1) Flashcards
(178 cards)
1
Q
Ischemic stroke
A
arteries to the brain become narrowed or blocked
2
Q
two types of ischemic strokes
A
thrombotic stroke and embolic stroke
3
Q
thrombotic stroke
A
clot forms in the arteries that supply blood to the brain
4
Q
embolic stroke
A
clot forms elsewhere and travels to the arteries in the blood stream
5
Q
von willebrand disease
A
most common inherited bleeding disorder
ability to form a platelet plug
6
Q
type 1 von willebrand disease
A
most common, usually mild to moderate
7
Q
type 2 VWF
A
moderate to severe
8
Q
type 3 VWF
A
rare and severe
9
Q
factor XI deficiency
A
typically genetic, over 200 distinct pathogenic variants are known
10
Q
Hemophilia
A
bleeding disorder caused by deficiency in specific clotting factor
X linked recessive
11
Q
Hemophilia A
A
deficiency in clotting factor VIII
12
Q
Hemophilia B
A
deficiency of clotting factor IX
13
Q
Pathogenic bleeding
A
due to a genetic or use of a drug
can effect primary or secondary hemostasis
14
Q
drugs for pathogenic bleeding
A
antiplatelets and anticoagulants
15
Q
Stoke symptoms (5)
A
trouble with speaking/understanding
paralysis/numbness of the face, arm and leg
trouble with sight in one/both eyes
headache
trouble with walking
16
Q
stroke
A
blood supply to part of the brain is interrupted or reduced
17
Q
hemorrhagic stroke
A
a blood vessel in the brain leaks and breaks
18
Q
two types of hemorrhagic stroke
A
intracerebral hemorrhage
subarachnoid hemorrhage
19
Q
intracerebral hemorrhage
A
bleeding occurs in the brain
20
Q
subarachnoid hemorrhage
A
bleeding occurs in the space between the surface of the brain and the skull
21
Q
Hematopoiesis
A
Development of blood elements from bone marrow stem cells
22
Q
Transient ischemic attack (TIA)
A
temporary block of blood flow to your brain
23
Q
Myocardial Infarction
A
heart attack
clot occurs in coronary artery
24
Q
Cytokines
A
Small signaling proteins
25
Deep Vein thrombosis
only half people show signs and symptoms
swelling of the leg alone in the vein
pain/tenderness while standing or walking
26
Production of RBCs
Hypoxia --> Kidneys produce EPO
EPO --> Erythropoiesis in bone marrow
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Pulmonary embolism
clot that travels to the lung
started as DVT
shown on an angiogram
28
treatment for pulmonary embolism
heparin or coumadin
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Reticulocytes
Immature RBCs
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heparin
inactivates thrombin
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coumadin
interferes with vitamin K
prevents new clots from forming
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Erythrocytes
Mature RBCs
33
arterial thrombi
formed under high pressure
more platelet rich
34
venous thrombi
formed under low pressure
more fibrin rich
35
Virchow's triad
three factors that increase risk of pathogenic thrombus:
endothelial injury
abnormal blood flow
hypercoahulability
36
primary erythrocytosis
disorder of the bone marrow
Polycythemia vera
37
endothelial injury
main influence on thrombus formation in the heart and the arterial circulation
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endothelial injury is caused by
hypertension, hyperlipidemia, high glucose in diabetes mellitus, traumatic vascular injury
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abnormal blood flow
the state of turbulence and stasis rather than laminar flow
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abnormal blood flow is caused by
hyperlipidemia, aneurysm, MI, cardiac arrhythmia, immobility and paralysis
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hypercoagulability
increased tendency to develop blood clots due to an acquired or inherited factor
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two types of hypercoagulability
inherited (primary)
acquired (secondary)
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types of acquired hypercoagulability
cancer
smoking
pregnancy
medications such as heparin, estrogen
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types of inherited hypercoagulability
Factor V Lieden
Protein C or S deficiency
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Factor V lieden
point mutation in the gene for Factor V
causes resistance to the anticoagulant effects of activated protein C
46
examples of endogenous anticoagulants
antithrombin
Protein S
Protein C
Tissue factor pathway inhibitor
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antithrombin
inactivates factor IIa (thrombin) and Xa
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Protein S
cofactor for protein C
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Protein C
inactivates factors Va and VIIIa
50
Tissue factor pathway inhibitor
inhibits the tissue factor VIIa and Xa
51
coagulation
process of forming a blood clot in response to injury
52
Hemostasis process (3 steps)
1. vasoconstriction
2. formation of a platelet plug
3. formation of a clot (thrombus)
53
primary hemostasis
formation of a weak platelet plug
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secondary hemostasis
formation of a thrombus which helps stable the platelet plug
55
embolism
obstruction of blood vessel by a foreign substance
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thromboembolism
clot breaks off, travels through circulation and gets stuck in a small blood vessel
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fibrinolysis
breakdown of a clot
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what does damages tissue release and what does it do?
releases plasminogen activator (tPA)
converts plasminogen to plasmin
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what does plasmin do?
breaks down fibrin and dissolves the clot
60
coagulation cascade two pathways
1. extrinsic
2. intrinsic
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extrinsic pathways
external trauma that causes blood to leave the circulatory system (bleeding)
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intrinsic pathways
activated by trauma inside the vascular system
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extrinsic players
factor 7 and 10
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intrinsic players
factors 8-12
65
where do the extrinsic and intrinsic pathways converge?
at factor x
"common pathway"
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what does factor X do?
converts prothrombin to thrombin
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under normal physiological conditions, there is a balance between? what does the balance prevent?
procoagulant system and anticoagulant system
prevents excessive blood loss
68
normal platelets
150,000-450,000 per uL
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thrombocytopenia
low platelets ( less than 150,000)
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when there is less than 50,000 per uL of platelets,
bleeding occurs easily
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when there is less than 20,000 per uL
spontaneous bleeding can occur in the absence of injury
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examples of decreased platelet production
bone marrow disorders
liver disease
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examples of increased platelet destruction
drug associated immune thrombocytopenia
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example of platelet trapped in the spleen
splenomegaly (enlarged spleen)
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thrombocytosis
high platelets (over 450,000 per uL)
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what could thrombocytosis be due to?
cancer, removal of the spleen or genetic mutations
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formation of the platelet plug (3 steps)
1. platelet adhesion
2. platelet activation
3. platelet aggregation
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platelet adhesion
exposes collagen and VWF
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platelet activation
platelets undergo an irreversible change in shape to increase surface area and secrete granules
80
platelet aggregation
circulating fibrinogen binds to GP receptors on platelets and cross links them forming the platelet plug
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after the vessel wall is broken, what is the blood exposed to?
collagen and VWF
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what responds to the injury and what does it release?
neutrophils and macrophages
Platelet Activating factor
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Platelet activating factor
activates platelets and causes them to stick to the vessel wall at the site of injury
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what do activated platelets release?
ADP, serotonin and thrombaxone A2
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what is the end result of blood clotting?
vasoconstriction
86
when there is no injury, how does the intact epithelium prevent platelet activation?
separating the blood from collagen
secreting NO and prostaglandin I2
CD39
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what does NO and PGI2 do?
inhibit platelet aggrergation
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what does CD39 do?
breaks down ADP in the blood which would normally promote platelet aggregation
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Hemostasis
forming clots in the walls of damaged blood vessels and preventing blood loss while maintaining blood in a fluid state
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How does hemostasis stop bleeding?
constrict blood vessels and insoles platelets
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platelets
anucleate cells found in blood
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How does a break in the skin close again?
1. hemostasis
2. inflammation
3. proliferation
4. remodeling
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splenomegaly
enlarged spleen
higher risk of rupturing, can lead to life threatening bleeding
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hyperslenism
spleen is overreactive, removing too many blood cells too quickly
leads to cytopenia
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cytopenia
shortage of blood cells
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congestive splenomegaly
spleen becomes enlarged and engorged with blood due to impaired blood flow through the splenic vein
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infiltrative splenomegaly
enlarged spleen caused by foreign cells invading the spleen
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Lymphoblastic Lymphoma
clone of immature T cells that become malignant in the thymus
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Multiple Myeloma (MM)
malignant proliferation of plasma cells that infiltrate bone marrow
aggregate into tumor masses in skeletal system
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Non-hodgkin Lymphoma
diverse group of lymphomas linked to chromosome translocations
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Risk factors of non-hodgkins lymphoma
older, male, white, having immune disorders, etc.
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Burkitt Lymphoma
most common NH lymphoma in children
very fast growing tumor on jaws and facial bones
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Hodgkin Lymphoma
presence of Reed-Sternberg cells in the lymph nodes
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Prescence of Reed-Sternberg cells in the lymph nodes are necessary for
diagnosis but not specific to Hodkins lymphoma
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Symptoms of Hodgkin Lymphoma
fever, weight loss, night sweats, pruritus
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can adult Hodgkin lymphoma be cured?
yes with early diagnosis and treatment
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Malignant Lymphomas
diverse group of neoplasms that develop from the proliferation of malignant lymphocytes in the lymphoid system
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how are malignant lymphomas classified?
REAL classification
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the two major categories of malignant lymphomas
Hodgkins Lymphoma
Non-Hodgkin Lymphoma
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Lymphadenopathy
enlarged lymph nodes that become palpable and tender
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Two types of Lymphadenopathy
local and general
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local lymphadenopathy
drainage of an inflammatory lesion located near the enlarged node
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general lymphadenopathy
occurs in the presence of malignant or nonmalignant disease
114
most common way to detect a lymph node
ultrasonography
115
chronic leukemias examples
chronic myelogenous leukemia
chromic lymphocytic leukemia
116
chronic myelogenous leukemia
too many blood cells made in the bone marrow
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chronic lymphocytic leukemia
too many immature lymphocytes
slow progression
118
most common adult leukemia in the western world
chronic lymphocytic leukemia
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most common adult leukemia
acute myelogenous leukemia
120
acute leukemias examples
acute myelogenous leukemia
acute lymphocytic leukemia
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acute myelogenous leukemia
too many myeloblasts
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acute lymphocytic leukemia
too many lymphoblasts
mostly in children
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treatment for acute leukemia
chemotherapy
124
acute leukemias are manifested by
bone marrow depression, fever, anorexia, neurologic symptoms
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Leukemias
malignant disorder of the blood and blood forming organs
excessive accumulation of leukemic cells
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Pancytopenia
cells crowd bone marrow
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acute leukemia
presence of undifferentiated/immature cells
usually blast cells
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chronic leukemia
predominant cell is mature but does not function properly
129
Lymphocytosis
increase in the number of proportion of lymphocytes in the blood
includes acute viral infections: epstein barr virus
130
Lymphocytopenia
decrease in the number of circulating lymphocytes in the blood
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quantitative disorders
increases or decreases in cell numbers
bone marrow disorders/ response to infectious organisms
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leukocytosis
normal protective physiologic response to stressors
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leukopenia
not normal/beneficial
a low white count predisposes a patient to infections
134
polycythemia vera
rare cancer of the blood producing cells of bone marrow
mutation in the JAK2 gene
135
secondary erythrocytosis
due to increased EPO production
136
what can lead to overproduction of EPO?
chronic low oxygen levels can cause a compensatory increase in hemoglobin production
renal tumor
drugs (testosterone, Epotein)
137
erythrocytosis
increase in the production of RBCs leading to elevated hemoglobin
138
signs/symptoms of erythrocytosis
hypertension, frequent nosebleeds/bruising, pruritus
139
Sickle Cell Disease
2 alpha globin chains and 2 mutated beta global chains due to a non-conservative missence mutation
140
sickle cell carrier
has one copy of the gene and one normal gene
no health problems
reduced risk of malaria
141
autosomal recessive disease
two copies of the altered hemoglobin gene
SCD
142
Sickle cells can get stuck in small capillaries and lead to
vase-occlusion, ischemia and pain crisis
143
sickling of cells in SCD is due to
deoxygenated hemoglobin becoming aggregated --> loss of normal shape
144
how much folate can be stored in the liver?
1 month
145
how much B12 can be stored in the liver?
large quantities
3000-5000mcg
146
Sideroblastic anemias
rare disorder in which bone marrow produces abnormal RBCs called ringed sideroblasts
microcytic anemia
plasma iron levels are normal to high
147
pathophysiology of sideroblastic anemias
abnormal heme synthesis and inability to incorporate iron into heme
genetic/aquired dysfunction of one of the many enzymes in heme biosynthesis pathway (Shaman pathway)
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Thalassemias
genetic, microcytic anemia
abnormal or absent production of alpha or beta globin
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Alpha thalassemia
alpha globin gene deletion resulting in reduced or absent production
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beta thalassemia
point mutations in beta globin gene
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beta thalassemia minor
mild, asymptomatic
152
beta thalassemia intermedia
mild to moderate symptoms
153
beta thalassemia major
severe, complete absence of beta chains
lifelong blood transfusions
154
Hemochromatosis
iron overload occurs in the body, causing iron toxicity
storage of iron in the heart, liver, pancreas and causing organ failure
155
gene involved in hemochromatosis
HFE gene
affects pattern of iron absorption
156
ferritin
protein that stores iron
157
transferrin
protein that transfers iron in the blood
158
about how much dietary iron per day is required
10-20 mg
159
Porphyrias
heme proteins are constantly synthesized and degraded
cells do not convert porphyrins to heme in a normal manner
160
rate limiting step in porphyrias
succinyl CoA and glycine --> delta aminolevulinic acid
ALA synthase is the enzyme
161
symptoms of porphyria
formation of superoxide radicals leads to
skin damage from exposure to light
162
two erythrocyte disorders
anemia (low Hb)
erythrocytosis (high Hb)
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two leukocyte disorders
leukopenia (low WBC)
leukocytosis (high WBC)
164
two thrombocyte disorders
thrombocytopenia (low platelets)
thrombocytosis (high platelets)
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two disorders of coagulation
hemorrhage (excessive bleeding)
thrombosis (blood clot)
166
hematopoiesis
development of the formed elements of blood from bone marrow stem cells
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hematopoietic cells are found in the
peripheral blood and in bone marrow
168
reticulocytes
immature RBCs
make up 1-2% of circulating RBCs
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erythrocytes
mature RBCs
circulate for 120 days and destroyed by macrophages
170
what has a higher affinity for heme than oxygen?
CO
171
at higher elevations, what has a higher affinity to heme?
myoglobin because there is less oxygen so more is trying to go to the tissues
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myoglobin has a higher binding affinity in the _____ while hemoglobin has a higher binding affinity in the ______
tissues
lungs
173
hemoglobin has a ______ binding affinity for oxygen at high pressure
even lower
174
what regulates the binding and release of oxygen in Hb
allosteric interactions
175
does the mother or baby have a higher affinity for oxygen?
the baby
176
fetal hemoglobin
2 alpha and 2 gamma chains
177
difference between hemoglobin and fetal hemoglobin
fetal hemoglobin has a serine residue instead of histidine
reduction of 2 positive charges
reduced affinity for 2,3 BFG and increased affinity for oxygen
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the lower the P50,
the higher the binding affinity
