Blood wk 2, clinical Flashcards

1
Q

what does a deficiency in factor 8 cause?

A

Hemophilia A

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2
Q

who is more commonly effected by hemophilia A?

A

males (on X chromosome)

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3
Q

what is the difference between mild, moderate and severe hemophilia?

A

mild: 49%
moderate: 6-49%
severe: less than one (could severely bleed at any time)

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4
Q

which is more likely hemophilia A or B?

A

A is 4x more common

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5
Q

what occurs with hemophilia A?

A

most people only experience issues after major bleeding event like tooth extraction or surgery

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6
Q

what are the genetics of hemophilia?

A

X inked recessive
(effected males will pass to a daughters as carriers, not sons)
30% cases spontaneous

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7
Q

what is the treatment for hemophilia A?

A

DDAVP- mild cases
Factor 8 replacements

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8
Q

what is the newest treatment for hemophilia?

A

roctavian treatment gene therapy (using virus vectors)

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9
Q

what causes hemophilia B?

A

factor IX

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10
Q

what is the treatment for hemophilia B?

A

routine treatment regimen of intravenous infusions of Factor IX
replacement products to maintain sufficient levels of clotting factor to prevent bleeding episodes

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11
Q

what is the treatments for bleeding patients with vit K deficiency?

A

fresh frozen plasma (also subcutaneous vit k)

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12
Q

what is hemgenix?

A

one-time gene therapy product given as a single dose by IV infusion

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13
Q

what do you do for a patients with supratherapeutic INR values due to being on warfarin and an antibiotic?

A

1- make sure not bIeeding
2-INR is >5, warfarin
should be withheld for at least 1 dose, and close follow-up
monitoring should be arranged

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14
Q

what is something to be aware of for patients taking warfarin?

A

need to keep a constant diet, too much K can throw everything off

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15
Q

what s the cheapest simplest thing to do for someone bIeeding and need of quick correction?

A

fresh frozen pIasma

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16
Q

what is the mechanism of warfarin?

A

vit K epoxide reductase inhibitor (keeps vit K reduces leading to inactive clotting factors)

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17
Q

what is the site of action for heparin?

A

anti-thrombin III

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18
Q

what is the site of action for warfarin?

A

vit K epoxide reductase

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19
Q

what does heparin do?

A

reduction in activity and synthsis of thrombin

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20
Q

can you use heparin or warfarin during pregnancy?

A

heparin yes
warfarin no

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21
Q

what is von wiIIiebrand disease?

A

vWF stored in Weibel-Palade bodies of endothelial cells & alpha granules of megakaryocytes/platelets

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22
Q

what is the genetic inheridance of von wiIIiebrand?

A

autosomaI dominant

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23
Q

why is there a cIotting issue seen with VWB?

A

because the first step of co-ag cant happen, pIateIets can not ahere to the waII

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24
Q

what is thrombocytopenia?

A

low platelet count (beow 150,000)

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25
what are the major pathophysiologic mechanisms of thrombocytopenia?
* Decreased platelet production in the bone marrow * Peripheral platelet destruction by antibodies * Consumption in thrombi * Sequestration (pooling) of platelets in the spleen in individuals with portal hypertension and/or splenomegaly
26
what are some common causes of thrombocytopenia?
* Primary immune thrombocytopenia (ITP) * Drug-induced immune thrombocytopenia * Hypersplenism due to chronic liver disease -cancer -hereditary -aplastic anemia
27
what is idiopathic thrombocytopenic purpura or ITP?
bruising; petechiae, nosebleeds and bleeding gums may occur if the platelet count is below 20,000, compared with a normal range of 150,000-400,000/mm
28
The most common specific factor inhibitors are
FVIII and FIX
29
antiphospholipid antibodies proIong PPT but are associated with:
increase in thrombosis rather than bleeding
30
Plasma comprises ____ of total volume of whole blood
~55%
31
what is serum?
If whole blood is allowed to contact glass, it will clot. After centrifugation, the fluid portion is SERUM. SO essentially same as plasma BUT NO clotting factors
32
Pre-analytical variables account for what percent of Iab errors?
Account for up to 75% of laboratory errors
33
what is lipemia?
milky plasma after fight fat meal
34
what is hemolysis?
rupture of pure RBC -could happen due to poor collection -releases cellular contents into blood (significant inc in K, Mg, Ph)
35
lipid panes can show:
-total cholesterol -HDL-C -LDL-C Triglycerides (VLDL)
36
what is a basic metabolic panel?
Multiple chemistry tests are grouped and reported as a single profile for ease of ordering. Includes electrolytes and tests of kidney function Na, K, C, CO2, BUN, Cr, Glu
37
what is the main determinant of plasma osmolality?
Na
38
what can too high of Na cause?
hypernatremia dehydration
39
what can decreased serum sodium cause?
hyponatremia increased fluid retention
40
what is the main intracellular cation?
Potassium
41
what can elevated K cause?
hyperkalemia renal insuffiencey cardiac conduction issues poor phlebotomy
42
what can decreased K cause?
hypokalemia diarrhea, diuretics
43
What is the major extracellular anion?
Chloride
44
how is chloride level usually changed?
usually in shift in Na or bicarb to maintain electrical neutrality
45
Serum Creatinine is excreted by the kidneys and so blood levels are therefore proportional to:
renal excretory function
46
why is blood glucose level important to monitor?
diabetes mellitus
47
What does hemoglobin A1c measure?
what percentage of hemoglobin is non-enzymatically glycated – correlates with average glucose level in the blood over lifespan of RBCs analyzed (2-3 months)
48
what does the total protein level reflect?
nutritional status liver disease and kidney function
49
What is albumin?
major protein in the extracellular portion of blood
50
what is albumin level important for?
maintains colloid osmotic pressure
51
what is the difference of indirect and direct bilirubin?
--Direct: Water-soluble conjugated bilirubin secreted into bile --Indirect: Insoluble Unconjugated circulating from RE system bound to albumin
52
what is total Ca?
The total serum calcium is a measure of both – Free (ionized) calcium – Protein bound (usually to albumin) calcium
53
what is alkaline phosphate?
increased when bile duct is blocked or cancer spreads to bones
54
what is the Alanine Aminotransferase (ALT) test?
An ALT blood test measures the amount of ALT in the blood to help diagnose or monitor liver disease α-ketoglutarate + alanine ⇌ glutamate + pyruvate
55
what is the Asparate Aminotransferase (AST) test?
shows Liver injury or irritation, heart attack or heart failure, kidney or lung damage, mononucleosis, or some types of cancer
56
what does the pancreatic serum amylase and lipase test show?
When the pancreas is damaged, these digestive enzymes can be found in the blood at higher levels than normal
57
what is the most abundant WBC?
neutrophils lymphocytes monocytes eosinophils basophils
58
what is average RBC count in male and female?
M: 4.7-6.1 F: 4.2-5.4
59
what is normal hemoglobin count in m and f?
m: 13.5-17.5 f: 12-15.5
60
what condition is marked by decreased WBC?
leukopenia
61
what condition is marked by increased WBC?
leukocytosis
62
what condition is marked by decreased platelets?
thrombocytopenia
63
what condition is marked by raised platelets?
thrombocytosis