Hematology Flashcards
(273 cards)
1
Q
What is the composition of plasma?
A
Water (92%)
Nutrients such as glucose, proteins, fats, and electrolytes
Contains albumin and other transport proteins, immunoglobulins, clotting factors, and enzymes
Contains small amounts of dissolved CO2 and oxygen (not bound to hemoglobin)
2
Q
What is plasma serum?
A
Plasma minus the clotting factors; specimen is not antio-coagulated
3
Q
What are acute phase reactants?
A
defined as proteins that change their serum concentration by >25% in response to inflammatory cytokines (IL-1, IL-6, TNFα)
4
Q
What system are the acute phase reactants a part of?
A
Innate immune system
5
Q
What can APRs play a role in mediating?
A
Fever, leukocytosis, thrombocytosis, increased cortisol, decreased thyroxine, and decreased serum ion
6
Q
Positive APRs
A
C reactive protein Fibrinogen (ESR) complement globulin D-dimer prothrombin factor VIII vW factor ferritin hepcidin ceruloplasmin haptoglobin alpha-1 antitrypsin platelets
7
Q
Negative APRs
A
albumin pre-albumin transferrin antithrombin transcortin retinol-binding protein transthyretin
8
Q
What lab test is used as a marker for inflammation in the body?
A
ESR
9
Q
White blood cells include?
A
Granulocytes, Monocytes, and Lymphocytes
10
Q
What are the three granulocytes?
A
Neutrophils, eosinophils, and basophils
11
Q
What are platelets derived from?
A
Megakaryocytes
12
Q
What are band cells?
A
Immediate precursor to the mature granuloctyes
13
Q
What is the main component of RBCs?
A
Hemoglobin
14
Q
4 pyrrole rings equals what?
A
1 protoporphyrin complex
15
Q
1 protoporphyrin complex + 1 atom of reduced iron equals what?
A
1 heme unit
16
Q
4 heme units + 4 globulin polypeptides (1 heme unit per globulin polypeptide) equal what?
A
1 molecule of hemoglobin
17
Q
After separating from the iron ion, the cleaved protoporphyrin complex gives rise to what?
A
bilirubin
18
Q
What is a CBC?
A
Complete Blood Count- automated reading in the lab that gives numbers and percentages of cells, quantifies RBC size, shape and color among other indices
19
Q
What is the most abundant blood cell type?
A
RBC
20
Q
Where are microcytic cells seen?
A
Iron deficiency and thalassemia
21
Q
Macrocytic cells are seen when?
A
BAD Hemoglobin Level B12 and folic acid deficiency Alcoholism Down syndrome Hypothyroidism Liver disease
22
Q
What is RDW?
A
Red cell distribution of width. If the number is high, it suggests a divergent population of red cells of different sizes
23
Q
What is anisocytosis?
A
RBCs of different sizes
24
Q
If you have pure macrocytosis what will your MVC and RDW values be?
A
Elevated MVC, Normal RDW
25
Iron deficiency anemia, what will your MVC and RDW values be?
Low MVC, Normal RDW
26
Mixed macroctyosis and microctyosis, what will your MVC and RDW values be?
Normal MCV, Elevated RDW
27
Hemolytic anemia with reticulocytes, what will your MVC and RDW values be?
Elevated MCV, Elevated RDW
28
Ovalocytes and elliptocytes characterize some inherited red cell abnormalities, what disease are these seen in?
hereditary elliptocytosis
29
Spheroctyes are seen in what diseases?
hereditary spherocytosis and autoimmune hemolytic anemia.
30
Schistoctyes are found when?
point to destruction within the vascular spaces. these are seen in hemolytic anemia, thrombotic thrombocytopenic purpura (TTP), disseminated intravascular coagulation (DIC), or a prosthetic heart valve.
31
Tear drop cells are found where?
in extramedullary hematopoiesis (EMH)
32
extramedullary hematopoiesis (EMH) comes into play when?
bone marrow failure.
33
EMH occurs in what conditions?
hemoglobinopathies, myeloproliferative disorders, or bone marrow infiltration by tumors
34
EMH occurs in what parts of the body?
spleen, liver, and occasionally the lymph nodes. less common organs; pleura, lungs, gastrointestinal tract, breast, skin, kidneys, and adrenal glands
35
EMH is part of what system of the body?
Reticular endothelial system
36
Macrocytic anemia will be shown in patients with what kind of deficiency?
Vitamin B12
37
Schistocytes are seen in what time of anemia?
Microangipathic hemolytic anemia
38
In hereditary spheroctyosis, there is a lack of what protein?
spectrin
39
What kind of protein is spectrin?
a key RBC cytoskeletal membrane protein
40
What test can be given in the lab to prove hereditary spherocytosis?
increased osmotic fragility
41
What do rouleaux RBCs look like?
stacked together long chains
42
When are rouleaux seen?
with increased serum proteins, fibrinogen and globulin. this is the mechanism for the erthrocyte sedimentation rate
43
what is the erthrocyte sedimentation rate for?
increases specifically with inflammation and increased "Acute phase" serum proteins
44
what are reticulocytes?
normal, immature form of the RBC. they develop and mature in the bone marrow and then circulate for about a day in the blood stream before fully maturing into RBCS
45
instead of a cell nucleus, what do reticulocytes have?
residual ribosomal RNA in their cytoplasm
46
what are howell jolly bodies?
rbc inclusion. inclusions of nuclear chromatin (DNA) remnants
47
what is basophillic stippling?
small dots at the periphery of red cells. the dots represent ribosomes
48
in what conditions is basophillic stippling seen?
lead and heavy metal poisoning, thalassemias, and alcohol abuse
49
what are heinz bodies and when are they seen?
denatured hemoglobin seen in G6PD deficiency
50
when are nucleated RBC seen?
bone marrow stress
51
what is one white blood cell inclusion?
dohle bodies
52
what are dohle bodies?
light, blue-gray oval, basophillic inclusions located in the peripheral cytoplasm of neutrophils
53
what are dohle bodies remnants of?
rough endoplasmic reticulum
54
when you see a high amount of poikilocytes, anisocytes and rbc inclusions, what does this usually mean?
spleen is not present
55
how many lobes in a neutrophil?
3-4 lobes
56
how many lobes in a eosinophil?
bilobed
57
how many lobes in a basophil?
3 lobed but difficult to see
58
what is the most abundant type of granulocyte?
neutrophils
59
more than 4 lobes of a neutrophil means they are what?
hyper segmented
60
what is the least plentiful blood cell type
leukocytes (WBCs)
61
how do neutrophils defend the body and when?
defend by phagoctyosis and enzymatic lysis. during infection
62
when do eisonophils defend the body and how?
react in allergic reactions and parasitic infection. defend by phagoctyosis and enzymatic lysis
63
what do basophils produce?
histamine, heparin, and IL-4
64
how do basophils defend?
release of chemical mediators
65
what do monocytes become?
macrophages
66
what are lymphocytes responsible for?
antibody production, cell mediated immunity, innate immunity
67
when are immature neutrophils seen?
during bone marrow stress. more neutrophils are produced as seen with infection, pregnancy, recovery from bone marrow suppression, and or hemotolgic malignancies
68
how many lobes in a band cell
uni-lobar
69
5 or more lobes of a neutrophil?
hyper segmented
70
when are hypersegmented neutrophils seen?
in megaloblastic processes (b12, folate deficiency)
71
a very high WBC count (>50,000) that is not leukemia, is what?
leukomoid reaction
72
when do you see an increased number of eosinophils?
```
NAACP.
N- neoplastic
A- asthma
A- allergic reactions
C- collagen vascular disease (rheumatologic)
P- parasites
```
73
what is basophilia?
increase of basophils in a persons blood
74
when is basophillia seen?
basophilic leukocytosis; associated with leukemia
(chronic myelongenous leukemia)
hypersensitivty
inflammatory reactions
hypothyroidism
infects with; TB, some viruses, Helminthic (parasitic) infections
75
what do B cells do
scan the intracellular environment for foreign invaders
directly kill virally or bacterially infected cells
naturally eradicate cancer cells
activate and help other immune cells including other lymphocytes that either chaperone, ingest, or make antibodies against foreign invaders
can remember a foreign invader they encountered decades ago
76
natural killer cells
cells kill tumor cells and virally infected cells. scan the surface. allows them to hunt down and destroy cells that are infected or that have become cancerous.
77
atypical lymphocytes
larger than normal. large nucleoli. more cytoplasm.
78
when are atypical lymphocytes seen
infectious mononucleosis or leukemia
79
monocytes
largest cell in the blood. produced in the bone marrow and stored in the spleen.
80
what do monocytes become when they travel to the site of infection and enter tissues?
macrophages
81
what do monocytes do?
perform phagocytosis, antigen presentation, and cytokine production to kill microbes
82
what are platelets?
anuclear cells derived from megakaryocytes
83
second most plentiful type of blood cell
platelets
84
what type of substances do platelets release for wound healing?
vasoactive compounds (vasoconstrictors) and platelet chemotactic agents (cytokines)
85
antigens a and b are what kind of antigens
carbohydrate
86
2nd most important consideration in transfusion medicine
D (Rh) antigen
87
when should RhoGAM be given:
- after delivery of an Rh positive baby
- routine prevention of Ph immunization at 26 to 28 weeks
- maternal or fetal bleeding during pregnancy from certain conditions
- actual or threatened pregnancy loss at any stage
- ectopic pregnancy
88
blood components
PRBCs, platelets, and fresh-frozen plasma
89
risks of transfusion:
- transfusion reaction
- GVDG (graph vs. host disease: WBCs and antibodies in the donors blood attack the recipient)
- fluid overload (give furosemide)
- infection (Hep B, C, HIV, etc.)
90
Immature cell types
bone marrow problem
91
smudge cells
CLL. WBC in the process of death = apoptosis
92
what do blast cells usually show?
great physiological stress or tumor cells
93
when are smudge cells indicated?
in the presence of lymphocytic leukemia
94
definition of a blast cell
very immature cells with larger nuceli that contain nucleoli. indicative of acute lymphocytic leukemia
95
Low hb/hct indicative of:
anemia
96
anemia: Red cell loss
```
GI bleeding
menorrhagia
excess phlebotomy
blood donation
(high retic count)
```
97
anemia: inadequate production
```
(low retic count)
absence of stimulus to produce:
- low erythropoietin level in CVD
- hypothyroidism
- androgen deficiency
lack of/abnormal raw materials:
- folic acid
- vitamin b12
- Fe
- hemoglobinopathy
intrinsic bone marrow problem:
- myelofibrosis
- malignancy
- aplastic anemia
- poisons, drugs
- radiation
```
98
normal lifespan of RBC
110-120 days (4 months)
99
symptoms of anemia:
dyspnea at rest, fatigue, lethargy, confusion, CHF, angina, MI, and arrhythmia, and depend on both MAGNITUDE and the RATE OF DEVELOPMENT
100
in acute bleeding, what is the main problem?
hypovolemia
101
normal adult blood volume
~5L
102
blood volume according to age:
adult female: 65mL/kg
adult male: 70mL/kg
children: 80mL/kg
neonates: 100mL/kg
103
when does shock happen and what are the symptoms?
when supply cannot meet demand. symptoms; confusion, dyspnea, diaphoresis, frank hypotension leading to permanent organ damage and possibly death
104
CBC:
H/H and red cell indices: MCV, MCH, MCHC, and RDW
105
reticulocyte count:
should be high if marrow is intact and responsive; if low look for problems in the marrow that impact cell production
106
WBC:
If low, consider bone marrow suppression; if high, consider infection, chronic inflammation or malignancy. Check for hypersegmented PMN’s whenever RBCs are macrocytic and reduced in number.
107
platelets
- If low, consider bone marrow suppression, hypersplenism, alcoholism, autoimmune states;
- If high, consider iron deficiency, inflammation, infection, malignancy, stress
108
microcytic anemia:
MCV <80fL
iron deficiency, chronic blood loss, alpha or beta thalassemia minor, and anemia of chronic disease/inflammation; lead poisoning
109
macrocytic anemia
MCV >100fL
B12, folate deficiency, alcohol abuse, Down syndrome, hypothyroidism, liver disease; also drugs (hydroxyurea, AZT), myelodysplasia, leukemia, reticulocytosis
110
Normocytic anemia
Acute blood loss, early iron deficiency, anemia of chronic disease, bone marrow suppression, chronic renal insufficiency, hypothyroidism, aplastic anemia, sickle cell anemia
111
iron deficiency anemia causes what most commonly
microcytic-hypochromic anemia
112
usual suspects of iron deficiency anemia:
NSAID use, gastric ulcers, heavy menses, colon cancers, blood donation; low dietary intake in pregnant women and children
113
an enzyme ______, on the brush border of the ________ and ________ reduces iron from ferric to ferrous making it more absorbable
- ferric reductase
| - duodenum and proximal jejunum
114
most of the iron in the body is found where
hemoglobin
115
where are smaller percentages of iron found?
ferritin and hemosiderin
116
very small amount (3 mg) is present in the transport form which is?
attached to transferrin
117
iron is transported bound to a molecule called
transferrin
118
what measures transferrin levels
total iron-binding capacity (TIBC)
119
what is the main storage form of iron?
ferritin
120
labile form of iron storage
ferritin
121
stable form of iron storage which consists of ferritin and cell debris
hemosiderin
122
diagnostic test for iron deficiency
ferritin
123
indirect marker of the total amount of iron stored in the body
plasma ferritin- diagnostic test for iron deficiency
124
are platelets an acute phase reactant
yes
125
treatments for iron deficiency
- 325 mg ferrous sulfate po tid x 6 months w/ Vit C
| - Ferrous gluconate 240/324 mg might be better tolerated
126
lead interferes with the biosynthesis of what?
heme
127
desire to eat non-nutritive things
pica
128
ingestion of lead
plumbophagia
129
compulsive consumption of ice
pagophagia
130
most common form of adult anemia
anemia of chronic disease
131
what are reduced during anemia of chronic disease
serum ion and transferrin saturation
132
Cytokines released in inflammatory and infectious disorders induce excess synthesis of what?
hepcidin
133
what is the primary intrinsic regulator of anemia of chronic disease
hepcidin
134
hepcidin, through the down-regulation of what it inhibits transfer of stored intracellular iron back into plasma, thereby blocking reuse of iron by the marrow
ferroportin, a transfer protein
135
levels of transferrin in fe def.
elevated
136
levels of TIBC in fe def
elevated
137
levels of ferritin in fe def
low
138
levels of transferrin in anemia of chronic illness
low
139
levels of TIBC in anemia of chronic illness
low
140
levels of ferritin in anemia of chronic illness
increased
141
structural abnormalities in the globin
hemoglobinopathies (qualitative)
142
underproduction of normal globin subunits
thalassemias (quantitative)
143
Caused by a genetic defect that results in abnormal structure of one of the globin chains of the hemoglobin molecule
hemoglobintopathy
144
what kind of traits are hemoglobinopathies inherited as
autosomal co-dominant traits
145
sickle cell disease is what?
autosomal recessive disease that leads to hemolytic anemia
146
sickle cell mutation
HbS
147
when do sickle cell symptoms begin
when HbF production ends
148
what creates painful crises lasting hours to days exacerbated by dehydration, acidosis, hypoxemia
vascular occlusions
149
salmonella osteomyelitis
if you get some salmonella in your GI tract you may not get sick but if it ends up in your bones and you have sickle cell- you are more likely to get infected and get sick. These only happens when you auto splenectomies your spleen.
150
what kind of cells will you see in a sickle cell disease smear?
normochromic RBC’s, nucleated RBC, sickle cells, Howell-Jolly Bodies, Retic count increased, increased bilirubin
151
short term sickle cell treatment
pain control, IVF in acute crisis (medically lowering the hematocrit and making the blood more viscous), oxygen
152
long term sickle cell treatment
```
transfusion
red cell exchange aphaeresis
hydroxyurea (promotes HbF production)
PCN prophylaxis- prophylax with penicillin
pneumoccal vaccination
folate supplementation
avodiance of smoking
iron chelators
```
153
thalassemias
hereditary microcytic aenmia; unresponsive to Fe
154
Alpha-Thalassemia in what type of ancestry
asian
155
Beta-Thalassemia in what type of ancestry
mediterranean
156
Due to a deletion or mutation in one or more of the four alpha globin gene copies.
Alpha thalassemia
157
microcytic, hypochromic anemia with low MCV.
Alpha Thalassemia Trait
158
what can HbH disease cause?
moderate to severe anemia and splenomegaly
159
Hydrops fetalis (fetal congestive heart failure) = Bart’s Anemia
barts anemia
160
hydrops fetalis
congestive heart failure (fetal) swollen appearance of the patient.
161
what is beta thalassemia caused by?
polymorphic mutations in one or both of 2 beta globin (HB ß) genes
162
Beta Thalassemia Trait (Thalassemia Minor)
one normal gene and one with a mutation; hypochromic, microcytic; unresponsive to iron
163
Beta Thalassemia Intermedia
two abnormal genes but still producing beta globin unit variety(ies) with some functionality; the severity of the anemia and health problems experienced depend on the mutations
164
Beta Thalassemia major (Cooley’s anemia)
two (very) abnormal genes; no beta globin produced; no Hgb A
165
when does cooleys anemia appear
in an infant after 3 months of age after loss of Hgb F; life threatening anemia
166
atypical, abnormal nucleated erythroblasts with granules of iron accumulated in perinuclear mitochondria
sideroblasts
167
Sideroblastic Anemia
The body has iron available but cannot incorporate it into hemoglobin = ineffective erythropoiesis
168
causes of sideroblastic anemia
```
B6 (pyridoxine) deficiency
Hereditary; isoniazid
INH use
Lead Poisoning
Zinc Poisoning
```
169
Mnemonic for Microcytic Anemia
```
T- thalassemia
A- anemia of chronic disease/inflammation
I- iron deficiency
L- lead poisoning
S- sideroblastic anemia
```
170
bound in the duodenum to intrinsic factor, which is produced by gastric parietal cells of the stomach. The complex is then absorbed in the terminal ileum (receptors that can bind to the intrinsic complex B12) and stored in the liver
vitamin b12
171
inadequate b12 levels are due to what?
dietary deficiency or deficient absorption
172
deficient b12 absorption is found in what?
```
crohns disease
short bowel syndrome
cystic fibrosis
bacterial overgrowth syndrome
pernicious anemia
```
173
type of b12 deficiency caused by lack of intrinsic factor
pernicious anemia
174
what causes the lack of intrinsic factor
- atrophic gastritis- achlorhydria: destruction of gastric parietal cells
- anti- intrinsic factor antibodies
- gastric bypass
- gastrectomy
175
water without chloride. your stomach doesn’t make enough stomach acid
Achlorhydria
176
most important system to absorb B12
intrinsic factor
177
more sensitive method of screening for B12 deficiency is measurement? why?
serum methylmalonic acid and homocysteinem levels. because they are increased early in b12 deficiency
178
what else is elevated in B12 deficiency
MMA and homocysteine
179
what is elevated in both b12 and folate deficiency
homocysteine
180
what are hypersegmented PMNs
white cell changes
181
if you give both a homocysteine test and MMA and only MMA is high, what is the deficiency?
folic acid problem
182
if you give both a homocysteine test and MMA and both are high, what is the deficiency?
B12 deficiency
183
neurologic problems
b12 deficiency
184
important to the formation of fetus brain
folic acid deficiency
185
Paresthesias- numbness or tingling
b12 deficiency
186
Big cells in the bone marrow
megaloblastic anemia
187
Big cells that get into the blood stream
macrocytic
188
causes of folate deficiency anemia
poor intake, pregnancy, ETOH, MTX
189
hypersegmented PMNs
folate deficiency anemia
190
what kind of cells can you see in folate deficiency anemia
howell-jolly bodies
191
where are megaloblasts found?
RBC precursors found in the bone marrow
192
where are macroctyes found?
in the peripheral blood stream
193
megaloblastic-macrocytosis can be caused by?
Folate and B12 deficiency
194
Intrinsic hemolytic anemia
hemoglobinopathies, some thalassemias, spherocytosis (osmotic fragility test), elliptocytosis, paroxysmal nocturnal hemoglobinuria, sickle cell disease; G6PD deficiency
195
Extrinsic hemolytic anemia
autoimmune, drug induced, HUS (hemolytic uremic syndrome), DIC (disseminated intravascular coagulopathy), metallic heart valves, infection, burns, hypotonic hemodilution
196
signs of hemolytic anemia
decreased haptoglobin
197
inclusions of denatured hemoglobin in the RBC found in G6PD deficiency
heinz bodies
198
bone marrow disorder where excessive RBC’s are produced (polycythemia rubra vera)
polycythemia
199
due to chronic hypoxemia (COPD), living at high altitudes
polycythemia
200
what can polycythemia lead to?
hyper viscosity
201
Normal MMA with elevated homocysteine suggests anemia secondary to?
pure folate deficiency
202
Low serum iron, elevated TIBC suggest?
iron deficiency
203
control of blood loss
hemostasis
204
first step in all wound healing
hemostasis
205
what does hemostasis do?
limits blood loss by precisely regulated interactions between components of the blood vessel wall, platelets, and plasma proteins
206
which natural set of anticoagulants keep hemostasis in check?
plasmin system and proteins C, S, and Z
207
what can unregulated activation of hemostasis cause?
thrombosis and embolism
208
what are the 3 major steps in hemostasis?
1. vasoconstriction
2. temporary blockage of break in the wall of a blood vessel by a platelet plug vis platelet adhesion
3. formation of a fibrin clot
209
what happens during vasoconstriction during hemostasis?
the blood vessel will automatically contract to stop blood loss. Nervous system tells muscular wall to contract in order to slow the bleeding.
210
what happens during temporary blockage of a break in the wall of a blood vessel?
Platelets will start getting sticky and they will aggregate and adhere to one another to make a platelet plug. They don’t stay here for long but they are fast.
211
what happens during formation of a fibrin clot?
Fibrin is a plasma protein and a final step in the activation of a clotting cascade. There are 13-14 clotting factors. Fibrin is like a fiber that criss crosses and form a mesh in every direction. First it is loose then the clot matures and the mesh tightens and it forms a solid plug.
212
In the normal state, the endothelial cells of intact vessels prevent blood clotting with a heparin-like molecule called?
thrombomodulin
213
what does thrombomodulin do?
inhibits thrombin (clotting factor IIa)
214
When endothelial injury occurs, the endothelial cells slow secretion of thrombomodulin and increase secretion of what?
von Willebrand factor
215
what does von Willebrand factor initiate?
platelet adhesion
216
Factor II
prothrombin
217
Activated factor II
thrombin
218
primary hemostasis
platelet plug
219
secondary hemostasis
plasma coagulation system that results in fibrin mesh formation—starts within 20 sec of injury. Makes a better clot. Step 3.
220
Thrombocytosis (aka thrombocythemia)
too many platelets. excessive clotting or bleeding.
221
Thrombocytopenia
Not enough platelets- can only result in bleeding
222
Von Willebrand’s disease
(defective adhesion; not really the platelets’ fault)
you have some sort of defect adhesion of the platelets but the platelets are too many or too few, its that the factor is not there or doing its job so it doesn’t make the platelets sticky like it should. The lack of or abnormality of the Von Willebrand adhesion.
223
Tiny, microscopic bleeding capillaries that form spots. Enough in the skin take a lot of the surface of the skin. Can form little purple spots known as purpura. Purpura can form bigger patches known as ecchymosis- black and blue
Petechiae, some purpura
224
bleeding time and platelet count
Abnormal platelet count or the bleeding test will have an abnormality. Not great tests available for platelet function but for the count we do.
225
Local measures generally suffice to control bleeding
Putting pressure on the bleed
226
Thrombocytosis
Platelet count above 500k/microliter. 100,000-450,000 is normal count.
227
what can cause thrombocytosis/thrombocypenia?
Can be caused by an acute reactive process (an APR: recent surgery, bacterial infection, iron deficiency, or trauma) or a bone marrow disorder which is a malignancy known as essential thrombocythemia)- cancer on platelets or megakaryocytes
228
treatment for thrombocytosis/thrombocypenia
- Treat underlying cause if possible (is it an acute phase reactant?)
- Antiplatelet medications, unless there is bleeding (next slide)
- Platelet-pheresis might be needed. Blood is removed from the pt. platelets are removed then blood is given back to the patient.
- hydroxyurea or anagrelide to lower platelet count in high-risk patients two drugs used to lower platelet count
229
irreversibly blocks the aggregation of platelets by interfering with the production of. Thromboxane (causes vasoconstriction) (prostaglandin- involved in fever, vasomotor tone)
aspirin
230
P2Y12 inhibitors (ADP receptor blockade)
block platelet aggregation
231
Thrombocytopenia
- decreased bone marrow production
- increased splenic sequestration: spleen isn’t allowing them to go into circulation
- accelerated destruction of platelets (ITP, TTP): increased destruction
232
Idiopathic Thrombocytopenic Purpura (acute)
immune mediated ITP is mostly seen in children with sudden onset of severe thrombocytopenia following recovery from a viral illness.
233
Idiopathic Thrombocytopenic Purpura (chronic)
mostly seen in adults. the antibodies never go away and keep attacking your platelets
234
Thrombotic Thrombocytopenic Purpura (TTP)
Initiated by vessel wall injury initiating an exaggerated clotting cascade leading to a tetrad of thrombocytopenia, hemolysis (red cells), renal failure, and CNS signs (central nervous system problems- mild to severe) such as fluctuations in levels of consciousness, confusion, and seizures
235
The only major clotting factor not made by the liver.
Von Willebrand Factor
236
what does the von willebrand factor bind to and protect?
inactive factor VIII from spontaneous degradation
237
what is responsible for the uncoupling of factor VIII from vWF
thrombin
238
what makes platelets sticky?
Free vWF
239
shearing stress
when the blood is rapidly flowing
240
treatment for von willebrands disease?
- vWF/factor VIII concentrate
- recombinant von Willebrand factor (r-vWF)
- desmopressin, which raises vWF. Given for surgery, major trauma, or other active bleeding.
241
primary site of synthesis of most of the clotting factors as well as the proteins involved in the fibrinolytic (keeps coagulation factor in check) system.
liver
242
body’s natural system
of anticoagulation that serves as a constant servo-
mechanism offsetting the coagulation cascade
The fibrinolytic system
243
liver pro-coagulation factors?
vitamin K-dependent: II (prothrombin), VII, IX, X (2,7,9,10)
| non-vitamin K-dependent: I (fibrinogen), V, XIII (1, 5, 13) basically all the other ones
244
liver anti-coagulation factors?
vitamin K-dependent:
proteins C, S, Z: made by the liver that interfere with the clotting cascade (not enough Vitamin-K)
non-vitamin K-dependent:
antithrombin-III and plasminogen
245
not synthesized by the liver and not vitamin K dependent
von willebrand factor (pro-coagulant)- vascular endothelial cells
thrombomodulin (anti-coagulant)
tPA (tissue plasminogen activator)- subendothelial tissue
246
important cofactor along many points in the coagulation cascade
Calcium (factor IV)
247
what are vitamin K dependent factors?
factors II, VII, IX, X, protein S, protein C, and protein Z
248
what do you see in coagulation factor disorders?
large palpable ecchymoses and large, spreading, deep soft tissue hematomas
249
what makes the fibrin clot tighten up?
factor XII
250
Prothrombin time (PT)
Tests extrinsic pathway and final common pathway
251
Activated Partial Thromboplastin time (aPTT)
Tests intrinsic pathway and final common pathway.
252
Thrombin time
tests for quantitative (fibrinogen deficiency) or qualitative (dysfunctional fibrinogen) defect. Thrombin is added to the patient’s plasma in the lab. Time till clot formation begins is noted. Measure of how long it takes for fibrinogen to make a cross link clot. Measures from after Fibrinogen (factor I) and down to where the clot is formed
253
What is the most common inherited plasma coagulopathy?
Factor VIII Deficiency: Hemophilia A
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what does factor VIII deficiency cause?
hemophilia A
255
what is a common cellular coagulopathy?
Van B Disease
256
what does factor IX deficiency cause?
hemophilia B
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second most common inherited plasma coagulopathy
hemophilia B
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what is factor XI deficiency?
autosomal recessive. less severe form of hemophilia.
259
body’s natural system of anti-coagulation that keeps coagulation in check
plasmin system
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what is plasmin?
proteolytic enzyme that lyses fibrin clots
261
what does protein S do?
activates protein C with the help of thrombomodulin
262
what does protein C do?
inactivates factors Va and Vllla
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what does protein Z do?
inactivates Xa
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what does antithrombin do?
inactivates thrombin (IIa) and Xa
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what does thrombodulin do?
converts thrombin (IIa) into an anti-coagulant; helps protein S activate protein C
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what does tissue factor pathway inhibitor (TFPI) do?
inactivates VIIa
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what does warfarin do?
blocks the activity of Vitamin K
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what is used to monitor the anticoagulant effects of warfarin?
Prothrombin time
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naturally occurring mixture of varied chain lengths of glycosaminoglycans (GAGs) derived from porcine intestine
heparin
270
what does heparin activate?
antithrombin
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what does antithrombin inactivate?
factor Xa and also inactivates activated thrombin
272
Warfarin messes with?
Vitamin K (2,7,9,10)- if you get a drug that messes with Vitamin K, it messes with everything Vitamin K is needed for. Messes with both coagulant factors and anti.
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what does heparin bind to?
platelet factor 4 (PF4) which triggers the formation of anti-heparin antibodies
