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N361: Pathophysiology II > Module 7 Part 1 - Hematology > Flashcards

Module 7 Part 1 - Hematology Flashcards

1
Q

Sickle Cell Anemia (SCA)

A

a group of autosomal recessive disorders which manifests as an anemia with sickle or crescent shaped RBCs d/t presence of abnormal type of Hgb S

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2
Q

What sort of genetic inheritance pattern is SCA?

A

Autosomal Recessive

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3
Q

What is Hgb S associated with besides SCA?

A

Protection from cerebral malaria (common disease in Sub-Sahara Africa

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4
Q

What populations most frequently have SCA?

A

Frequency of the disease is high in Mediterranean and African Populations

30% of their populations have the trait (carrier or disease)

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5
Q

What is the rate for an African American carrying the SCA gene (Sickle Cell Trait) in the US? How many African American newborns have SCA?

A

1 in 12 (8%) carrier rate

1 in 500 have SCA

90000-100000 people with SCA exist in the US currently with 50-70% reaching ages 40-50 – so its not longer a death sentence

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6
Q

What is the only treatment with a chance to cure SCA?

A

Bone Marrow Transplant

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7
Q

What are some examples of Sickle Cell Disorders?

A

Sickle Cell Anemia

Sickle Cell Hgb C

Sickle Cell Beta Thalassemia Disease

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8
Q

Under what conditions does Hgb S Sickle?

A
  1. Vasodilation causing conditions: Low pH, high H+ concentration, Acid build up (acidosis)
  2. Low O2, High CO2
  3. Exercise
  4. Dehydration
  5. Infection (especially bacterial)
  6. Stress and Anxiety
  7. Fever
  8. Exposure to the cold and high altitudes
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9
Q

If a person has the SCA trait (is a carrier) how much of their Hgb will be Hgb S?

A

Heterozygous for the trait (Ss) = 40% is Hgb S

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10
Q

If a person has the SCA disease how much of their Hgb will be Hgb S?

A

Homozygous Recessive (ss) = 90% Hgb S and a greater chance for sickling

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11
Q

Can Hgb S return to its original shape?

A

Yes! with enough oxygenation it can

However, the more times it does this the less likely it is to return to shape on subsequent events

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12
Q

What kind of Hgb is high in newborns? What do we do if the newborn will have SCA?

A

Hgb F

Hgb F is all metabolized by 5-6 mo post-birth, so if they are homozygous recessive we give hydroxyurea to increase Hgb F production so it is not metabolized as quickly and delay the sickling further out

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13
Q

What will be the hereditary pattern for parents that are AA (no SCA trait) and SS (homozygous rec for the trait) be like for SCA?

A

100% chance of child being a carrier

0% chance of children having SCA or being non carriers

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14
Q

What will the hereditary pattern for parents that are AS and SS be like for SCA?

A

50% chance of child having SCA

50% chance of being a carrier for SCA trait

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15
Q

What will the hereditary pattern be like for SCA if both parents are Heterozygous recessive for SCA trait?

A

50% chance of having the trait/being a character for the kids

25% chance the kid will have SCA

25% chance the child is normal with no trait or disease

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16
Q

How does SCA incidence differ between gender?

A

It does not since it is not sex linked

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17
Q

Lifespan of a normal RBC

A

120 days

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18
Q

Lifespan of a sickle cell

A

15-20 days

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19
Q

What is the structure of an RBC with normal Hgb?

A

smooth surfaced, flexible, disk that can mold its shape to move through arterioles, capillaries, and venules

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20
Q

What is the structure of an RBC with Hgb S?

A

Rough textured, rigid, elongated crescent shaped RBC

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21
Q

RBCs with Hgb S can only ___ and __ a certain amount of times

A

sickle and desickle

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22
Q

The process of sickling is reversible at first, but cells become susceptible to …

A

permanent entrapment by the macrophage system of the spleen and liver (can lead to impaired filtering)

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23
Q

What does entrapment of sickle cells by the macrophage system cause?

A

Hemolytic Anemia (increased destruction of RBCs resulting in Hgb deficiency)

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24
Q

Manifestations of SCA

A

Slightly jaundiced from hemolysis (sclera yellow, urine dark)

Painful excruciating crises / intense pain caused by vascular occlusion during sickling episodes

Sickling occurs more so in certain areas

Hypoxia from systemic anemia

Serious bacterial infection d/t inadequate splenic filtering of microorganisms

Potential Splenomegaly

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25
Q

What causes the Jaundice of SCA?

A

The destruction of RBC (since they have a shorter lifespan) leads to higher bilirubin levels causing jaundice, yellow sclera, and dark urine

Bilirubin can also cause brain damage

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26
Q

What is the cycle and cause of the painful excruciating crises in SCA?

A

VASCULAR OCCLUSION - which occurs from obstructions of sickled cells –> this decreased oxygen perfusion –> Which further increases sickling in the hands, feet, abdomen, back and joints causing intense pain from lack of oxygen

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27
Q

Where is sickling most apt to occur?

A
  1. Areas where blood flow is slowest (liver, spleen, kidney medulla)
  2. In tissue with high metabolic rate (brain (stroke) and muscle)
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28
Q

Systemic signs of anemia lead to …

A

hypoxia

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29
Q

Why is bacterial infection more likely in SCA?

A

Because the spleen gets inadequate filtering ability / filtering system damaged

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30
Q

Splenomegaly and SCA

A

As the spleen has to overwork and keep removing dead cells (potentially leading to acute crisis) –> progressive infarction from no blood occurs in the spleen leading to atrophy

Can just be enlargement or cause death and atrophy

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31
Q

What is the most common Hgb and healthiest in normal people?

A

Hgb A

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32
Q

Why are infants asymptomatic for SCA for 4-6 months?

A

It is due to presence of Fetal hemoglobin

Hgb F has a higher affinity for O2 than A

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33
Q

What is done for a diagnosis of SCA?

A

Step 1. Newborn screening for hemoglobinopathies are used to ID high risk individuals (via a stained blood smear to show sickle cells)

Step 2. Hgb Electrophoresis is used to ID presence of Hgb S and CONFIRM THE DISEASE

Step 3. Serial blood tests show blood abnormalities

Alternative 1: Can occur before 1, but prenatal testing can ID the presence of the homozygote state of the fetus

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34
Q

What is used to confirm SCA disease ?

A

Hemoglobin Electrophoresis

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35
Q

What may serial blood tests demonstrate in an SCA patient?

A

Decreased Hct

Decreased Hgb

Decreased RBC

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36
Q

Cure for SCA?

A

There is not really a cure, but you could try bone marrow transplant

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37
Q

What kind of drugs are available for SCA?

A

there are not really any drugs specifically for SCA other than Hydroxyurea

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38
Q

Hydroxyurea

A

Drug for SCA that increases the solubility of Hgb S - making the cell less likely to sickle

Given to babies who are positive for SCA or for treatment in adults

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39
Q

What is treatment like for Symptomatic SCA adults?

A

There is no compound to reduce or stop sickling and no special diet vitamin or iron therapy

So, it is a “band aid” approach that is mostly about prevention of symptoms or pain relief

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40
Q

What sort of treatments are given to people with SCA?

A

Pain relief requiring potent narcotic analgesics (Since SCA is very painful)

Transfusions during crisis, to promote healing post-ulcer, or during the last trimester of pregnancy

Pneumovax vaccinations

Prophylactic antibiotics (d/t spleen function impairment)

Increased hydrations during crisis

Hydroxyurea

Avoidance of precipitating factors

Bone marrow transplant

For parents: Genetic counseling

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41
Q

Potential Complications arising from SCA

A

Vaso-occlusive events

Splenic Sequestration

Stroke

Aplastic Crisis

Avascular Necrosis

Priapism in Males

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42
Q

What do Vaso-occlusive events cause?

A

tissue infarction with intense pain and disability

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43
Q

What does Splenic Sequestration cause?

A

Hypovolemia

Shock

Death

It is a plugged, enlarged, at greater risk for infection and potential rupture, and broken filter kidney

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44
Q

What can stroke lead to?

A

Weakness

Seizure

Inability to speak

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45
Q

Aplastic Crisis

A

Bone marrow needs blood flow to make blood, so it will stop functioning without it which can eventually lead to avascular necrosis

bone marrow temporarily stops erythropoiesis

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46
Q

Avascular Necrosis

A

Long bones of the leg or arm die from occlusion leading to a need for something like a hip replacement

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47
Q

Priapism

A

Males only

Painful prolonged penile erection lasting hours, days, or weeks d/t stasis and occlusion –> usually results in impotence if now extracted

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48
Q

Sickle C Disease

A

A sickle cell disease

Recessive Autosomal

Occurs later in life, with less severe painful crises and it occurs less frequently than SCA - this is d/t Hgb C being a little less prone to sickling

Not as severe as SCA

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49
Q

50% of Sickle C disease patients have episodes of what?

A

Abdominal or Musculoskeletal pain before age 10 and moderate anemia (Hgb 8-10 gm/dL)

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50
Q

What else is a Sickle C disease person at risk for?

A

Increased risk for infection and fever which can lead to crisis

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51
Q

What are some Complications from Any Sickle Cell Disease?

A

Splenomegaly

Cardiomegaly (less blood flow and working more causes diastolic problems)

Acute Chest Syndrome

Sickle Retinopathy

Decreased Vision

Pregnancy issues

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52
Q

Acute Chest Syndrome

A

Characterized by Atypical pneumonia resulting from pulmonary infarction (d/t no oxygen in the lungs)

Second leading cause of Sickle Cell hospitalizations

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53
Q

Sickle Retinopathy

A

Blockage of blood flow to the eyes leading to blindness from retinal detachment (60-70% of patients)

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54
Q

When is decreased visual acuity first noticed in sickle cell patients?

A

at 20-30 years of age

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55
Q

What are some potential pregnancy issues caused by Sickle cell disease?

A

Severe bone pain crises

high incidence of abortions, stillbirth, and neonatal death

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56
Q

___ is the key for sickle cell disease

A

Prevention

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57
Q

Hemophilia

A

disorder of blood clotting

It is a hereditary sex linked recessive disease resulting in either a deficiency of Factor VIII (A) or Factor IX (B)

It can be mild moderate or severe depending on clotting factor percentages

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58
Q

Types of Hemophilia and their prevalence?

A

A - “Classic hemophilia” - 80%

B - “Christmas Disease” - 20%

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59
Q

Hemophilia is very rare to find in ___

A

females

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60
Q

Prevalence of Hemophilia is ___ in ___ Male Births

A

125 in 1 million

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61
Q

Which clotting factor is missing in Hemophilia A?

A

Factor VIII

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62
Q

Which clotting factor is missing in Hemophilia B?

A

Factor IX - PTC - Plasmin Thromboplastin Component

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63
Q

What level of Factor VIII Clotting factor leads to Mild, Moderate, and Severe Hemophilia A?

A

Mild - 6-30%

Moderate - 2-5%

Severe - <1%

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64
Q

Children with SEVERE Hemophilia A…

A

rarely survive childhood, experience a life of pain if they survive, have immobility and social isolation as a result

<1% Factor VIII in these children

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65
Q

What chances for hemophilia A does a child have if the mother is a carrier and father does not have the trait?

A

25% chance of a normal boy; 25% chance of normal girl

25% chance of carrier female

25% chance of Diseased male

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66
Q

What chances for hemophilia A does a child have if the mother is a carrier and father does does have the trait?

A

25% chance of afflicted female; 25% chance of afflicted male

25% chance of carrier female

25% chance for normal male

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67
Q

What chances for hemophilia A does a child have if the mother is not a carrier but the father does have the trait?

A

50% chance for carrier female

50% chance for non affected male

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68
Q

What chances for hemophilia A does a child have if the mother is affected and father does not have the trait?

A

50% chance for carrier female

50% chance to have an affected male (100% of males are affected)

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69
Q

What is the only situation where a female can be born with hemophilia?

A

If the mother is a carrier and the father has the disease (25% chance)

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70
Q

What is the main manifestation of Hemophilia A?

A

Spontaneous or Excessive Bleeding and Hemarthrosis

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71
Q

What are some examples of spontaneous or excessive bleeding in a Hemophilia patient?

A

Severe episodes provoked by minor trauma

Fatal intracranial hemorrhages with minor head bumps

Deep hematomas especially along the fascia resulting in compartment syndrome

Hematuria, Hematemesis, Tarry stools

*Minor bumps can be permanently debilitating

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72
Q

Hemarthrosis

A

Bleeding into the joints

IN hemophiliacs, joint swelling, degenerative changes, pain, limited ROM, permanent disability especially in the elbows, knees, and ankles can all occur

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73
Q

Compartment Syndrome

A

This is when the hematoma causes muscle tissue to get between the muscle and fascia preventing stretch eventually causing the muscles to die and nerves to become compromised

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74
Q

Diagnostics for Hemophilia A

A

Look for PROLONGED PTT (Partial Thromboplastin Time) - checks for clotting

See a decreased Measurement of Factor VIII

Prenatal Testing

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75
Q

Treatment for Hemophilia A

A

Factor VIII administration from fresh frozen plasma concentrate or cryoprecipitate

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76
Q

Complications arising from Hemophilia A

A

Intracranial Hemorrhaging

Infection with HIV (pre-blood screening)

Very risky to perform surgery - even dental

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77
Q

Where does Factor VIII need to be before, during, and after surgery?

A

greater than 30%

administer Factor VIII to achieve this

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78
Q

90% of coagulation disorders relate to ___ ___

A

platelet dysfunctions

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79
Q

What is the first line of defense against accidental blood loss?

A

Platelets

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80
Q

How long do platelets circulate for?

A

Normally circulate for 8-10 days then are destroyed by macrophages in the liver and spleen

81
Q

At anyone one time ___ platelets are in slow transit in the spleen and do not figure into platelet count?

A

1/3 - only platelets in the blood matter

82
Q

What happens to platelet count if there is no spleen?

A

There is a higher platelet count since the normal 1/3 is now in circulation. (also higher risk for stroke, clotting, thrombus, and infection)

83
Q

Normal Platelet Count

A

150,000-400,000 cells per microliter

84
Q

Platelet count is usually higher in ___ patients

A

Splenectomized

85
Q

How does sympathetic input into the spleen impact platelet levels?

A

Stress –> release of EP –> splenic contraction –> release of platelets into circulation (potentially to deal with trauma)

86
Q

Platelet count is low in people with Splenomegaly, why?

A

There is increased trapping in slow transit (in the spleen) of platelet

OR

There is increased macrophage destruction of platelets occurring

87
Q

Classic Manifestation of Low Platelet Count

A

Petechiae

88
Q

What causes low platelet Petechiae?

A

Loss of normal endothelial plugging (does not mean deep hemorrhaging is occurring)

It means that the normal platelet cell wide block between normal endothelial blood cells is not blocked so RBC can leak through and cause the petechiae

89
Q

At what platelet count should the patient be on bleeding precautions (No IM, shaving, toothbrush, etc) for Severe and Rare spontaneous hemorrhaging?

A

Less than 20,000 cells per microliter

90
Q

Main 2 Types of Platelet Disorders

A
  1. Quantitative (Thrombocytopenia)
  2. Qualitative (Thrombocythemia)
91
Q

Thrombocytopenia

A

Decreased number of platelets (Quantitative disorder)

92
Q

Possible Responsible Mechanisms for Thrombocytopenia

A
  1. Decreased or defective platelet production in bone marrow
  2. Decreased platelet survival (Idiopathic Thrombocytopenia)- increased destruction outside the bone marrow caused by an underlying disorder
  3. Sequestration (pooling of blood in the spleen or increased amount of blood in a limited vascular area)
  4. Intravascular Dilution (With IV fluids lowering per unit volume)
  5. Blood loss - hemorrhaging
93
Q

The most common kind of Thrombocytopenia

A

Idiopathic Thrombocytopenic Purpura

94
Q

Idiopathic Thrombocytopenic Purpura

A

Autoimmune issue where the spleen becomes a site of immunoglobulins and increased phagocytosis of platelets

2 forms: Acute and Chronic

95
Q

Acute Idiopathic Thrombocytopenic Purpura

A

“Post Viral Thrombocytopenia”

Usually affects children between 2 and 6

Usually post virus where the antibodies can fit the virus AND the platelets leading to destruction

96
Q

Chronic Idiopathic Thrombocytopenic Purpura

A

“Essential or Autoimmune thrombocytopenia”

Usually affects adults < 50

Especially in women 20-40 yo

97
Q

Manifestations of Thrombocytopenia

A

Petechiae

Purpura

Ecchymosis (color changes)

Menorrhagia

Bleeding in the eyes (subconjunctival hemorrhage), ear pain, epistaxis, bleeding of gums, hemoptysis, frank or occult blood in stools, painful joints, mental status changes

98
Q

Petechiae

A

Small spots of subcutaneous bleeding

99
Q

Purpura

A

Large areas of subcutaneous bleeding

100
Q

Menorrhagia

A

Excessive menstruation

101
Q

Hemoptysis

A

Coughing up blood

102
Q

Frank Blood in Stools

A

Bright red blood in stools added past digestion in the stomach

103
Q

Occult Blood in Stools

A

Digested and Black stool from digested blood

104
Q

When is thrombocytopenia most common?

A

In children, usually preceded by viral infection - it is acute and usually resolves spontaneously

105
Q

Therapeutic Supports for Thrombocytopenia

A

Platelet Transfusions

Corticosteroids

Protection from Trauma

106
Q

What are some other causes for Idiopathic Thrombocytopenia

A

Megaloblastic Anemia

Chronic Alcoholism

Viral Infections like HIV

Ionizing Radiation exposure

Neonate Issues

Liver Cirrhosis

Lymphoma

Metastatic Cancer

107
Q

What sort of in neonate causes can lead to thrombocytopenia?

A
  1. Maternal drug ingestions
  2. Maternal immunoglobulin attacking fetal platelets
  3. congenital syphilis
  4. maternal viral infection
108
Q

Why does Liver Cirrhosis cause thrombocytopenia

A

spleen enlarged with blood not flowing through the damaged liver which can lead to increased macrophage destruction

109
Q

Why does Lymphoma cause Thrombocytopenia

A

Cancer of the lymphoid tissue enlarges the spleen so 80% of the platelets get trapped there

110
Q

Why does metastatic cancer cause thrombocytopenia?

A

Infiltration of bone marrow can stop creation of platelets and megakaryocytes

111
Q

Examples of Drugs known to Decreased Platelet Count

A

Cancer chemo

Thiazide Diuretics

Quinidine

Quinine

Digitoxin

Methyldopa

Sulfonimines

Gold

ASA

Heparin

112
Q

Diagnostics for Thrombocytopenia?

A
  1. PLT< 20,000 cells/microliters
  2. Mean Platelet Volume (Size) Decreases
  3. Bone marrow function (Abundant megakaryocytes)
  4. Humoral tests to measure platelet associated IgG levels (non specific)
113
Q

What can Mean Platelet Volume tell us?

A

It is the size of an average platelet

Bigger platelets are usually younger and aggregate better, while older ones are smaller

This may help provider determine whether to give platelet infusion or not

114
Q

What does a low MPV indicate

A

Older platelets that do not function as well

115
Q

What can raised IgG levels tell us on a humoral test for platelet associated IgG levels?

A

It is a non specific test, but it can give a clue toward immune mediated thrombocytopenia

116
Q

What is treatment like for Kids with thrombocytopenia?

A

It is more conservative treatment with support in the post-viral period

117
Q

What is treatment like for adults with thrombocytopenia?

A
  1. Prednisone
  2. Immunosuppressants (to stop the attack against yourself)
  3. Splenectomy if treatment is ineffective
  4. Immune globulins postoperatively
118
Q

Prednisone

A

Med given to thrombocytopenia patients

it suppresses phagocytosis, promotes capillary integrity, and enhances platelet production

119
Q

What is the problem with splenectomy for thrombocytopenia treatment?

A
  1. they become susceptible to infection and need their vaccines like Pneumovax
  2. Post splenectomy thrombocytosis can occur - prevent thromboembolic complications by giving prophylactic heparin
120
Q

What are immune globulins (given postoperatively) for regarding thrombocytopenia?

A

It raises platelet count quickly in a short period of time (in 1-5 days but only lasts 1-2 weeks) - short term fix

121
Q

Tjhrombocythemia

A

Quantitative platelet disorder

Increase in the number of platelets

It is associated with an increased risk for thrombosis and clotting of the vasculature

122
Q

What may be some complications that occur d/t Thrombocythemia?

A

Stroke

Myocardial infarction

Pulmonary embolism (seen via resp distress)

DVT

123
Q

When is DVT often seen?

A

in pregnancy

124
Q

What does DVT with no explanation mean?

A

It is a red flag for cancer

125
Q

Causes for Thrombocythemia

A

Iron deficiency anemia

Polycythemia vera

acute stress response

metastatic cancers

chronic inflammatory diseases

splenectomized patient

oral contraceptives (especially in smoking women)

126
Q

How does Anemia lead to Thrombocythemia

A

It causes bone marrow to go into high gear and make more RBC, WBC, and platelets

127
Q

How does Polycythemia Vera cause Thrombocythemia

A

overproduction of RBC and platelets occur

128
Q

What kind of things can lead to an acute stress response responsible for causing Thrombocythemia?

A

Exercise

Post Partum

Trauma - s/p homorrhagia

Surgery

129
Q

What is important to look out for after surgery with thrombocythemia?

A

DVTs from increased clotting - look for warmth, deep pain, red color

could dislodge and become an embolus and lead to stroke or pulmonary emboli

130
Q

On day ___ post-op the patient is at highest risk for thrombocythemia

A

day 10

131
Q

What is an example of a chronic inflammatory disease leading to thrombocythemia?

A

Rheumatoid Arthritis

132
Q

How does metastatic cancer lead to thrombocythemia?

A

it increases bone marrow production

133
Q

Thrombocytopathy

A

Qualitative platelet disorder

Normal amount of platelets, but they do not aggregate like they should

134
Q

What can cause Thrombocytopathy?

A
  1. Drugs like ASA, antihistamine, and NSAIDS
  2. Chronic Renal Failure - toxic effects of retained urea saturated blood cells and inhibits platelet aggregation
135
Q

What can we use drugs like ASA, antihistamines, and NSAIDs for knowing it can decreases aggregation?

A

We can use it to lower thrombus risk by lowering aggregation

136
Q

There are ___ clotting factors

A

13

137
Q

What is the purpose of fibrin?

A

To work as cross bridges between platelets to stabilize and cause a platelet plug

138
Q

Platelet Aggregation Steps

A
  1. Vessel is damaged from some event - cascade initiated
  2. instantaneous accumulation at the site d/t smooth endothelium attracting platelets
  3. platelets release substances
  4. undamaged endothelium released prostacyclin and nitric oxide

5-final: clotting factor X makes prothrombin into thrombin which cleaves fibrinogen into fibrin

139
Q

What do platelets attach to in order to release Serotonin, ADP, and Other Chemicals?

A

Proteins - Von Willebrand Factors

140
Q

What does Serotonin do for bleeding?

A

causes vasoconstriction to decrease bleding

141
Q

What does Serotonin, ADP, and Other chemicals ultimately do for platelets?

A

it transforms the platelet slipper disc shape into sticky spheres that form the platelet plus

142
Q

Thromboxane A2

A

chemical released by platelets that attracts more platelets to an area

143
Q

What does Fibrinogen do?

A

It cleaves to fibrin and connects between exposed sites in platelets like a bridge –> thus stabilizing the plug

144
Q

To prevent unimpeded platelet aggregation, what must happen?

A

Undamaged endothelial cells release Prostaglandin I2 and Nitric Oxide

145
Q

What does Prostaglandin I2 (Prostacyclin) and Nitric Oxide do in the clotting cascade?

A

It causes vasodilation and inhibits platelet aggregation in areas where it should no aggregate

146
Q

Final step of the coagulation cascade

A

production of stabilized fibrin

147
Q

Coagulation reactions involve…

A

a series of 13 coagulation factors (proteins) that are activated in a domino manner which leads to coagulation (clotting) of the blood

148
Q

Coagulation Pathways?

A

Extrinsic Path

Intrinsic Path

Common path

149
Q

Extrinsic Pathway

A

normally coagulation occurs first through this pathway

this is activated by trauma to a blood vessel or surrounding tissue

Need extrinsic compounds found outside the blood to work

It is responsible for clot formation in response to tissue injury

150
Q

Intrinsic Pathway

A

A formation of a clot in response to an abnormal vessel wall in absence to vessel injury

Begins in circulation

151
Q

Common Pathways

A

When the intrinsic and extrinsic pathways merge at factor X

152
Q

Which clotting factor is responsible for converting the plasma protein prothrombin into thrombin

A

X (10)

153
Q

___ is the master regulator of the coagulation cascade and is the catalyst for fibrin that stabilizes the clot

A

Thrombin

154
Q

Which coagulation pathway is slower?

A

Intrinsic Pathway (begins in circulation as blood comes into contact with collagen in the injured vessel wall)

155
Q

Clotting Factors

A

Proteins that are synthesized in the liver

So, if there is a liver disease, there are less factors made

156
Q

Which pathway is effected by Hemophilia A and B?

A

Extrinsic Pathway (Factor 9 and 8 are in that path)

157
Q

Why do we give a Vitamin K shot at birth?

A

VitK is needed for clotting, and a newborn does not have the bacteria in the gut yet to produce it and they are a bleeding risk

158
Q

What clotting factors need Vitamin K?

A

Factors 7, 9, and 10 (and prothrombin)

159
Q

Coumadin

A

Anticoagulant

This interferes with the vitamin K clotting factors to prevent clotting

160
Q

How do we measure how much coumadin is needed?

A

we take a PT to know

Since Factor 10 helps prothrombin become thrombin it is PT viewed in Vitamin K factors

161
Q

Heparin

A

Anticoagulant

Works on Non Vitamin K Factors and uses PTT time to measure heparin therapy

162
Q

Both Heparin and Coumadin…

A

extend bleeding time

163
Q

Disseminated Intravascular Coagulation (DIC)

A

Clotting and Hemorrhaging occurring in the vascular system simultaneously

rare, but almost always fatal

happens everywhere in the body

It is basically a clotting event that spreads alongside hemorrhaging

164
Q

What does DIC cause?

A

Small blood vessel blockage

Organ tissue damage

Necrosis

Depletion of clotting factors and platelets

Activation of fibrinolysis which can lead to severe hemorrhaging

Organ impacts: kidney, brain, lungs, pituitary and adrenal glands, GI mucosa

165
Q

What will running out of the clotting factors and platelets cause in DIC?

A

no ability for endothelial plugging, eventually you will bleed from every oriface and death occurs (usually when the heart does not get enough blood)

166
Q

Why can we not give Heparin to a DIC patient

A

they are already bleeding! don’t extend that!

Also you cannot give clotting factors since that’s also already happening

167
Q

Causes for DIC

A

Infection (septicemial, viral, fungal, protozoal)

Obstetric Complications (abruptio placentae, post partum hemorrhage)

Neoplastic Disease (acute leukemia, metastatic cancer, lymphoma)

Disorders that produce necrosis (burns, transplant rejection, liver necrosis)

Shock

Transfusion reaction

DKA, PE, SCA, ARDS

168
Q

What is the pathophysiology pathway for DIC?

A

1 Coagulation System Triggered

  1. Excess fibrin forms and becomes trapped in microvasculature along with platelets –> leading to clots
  2. There is decreased blood flow to the tissue following a clot –> causes acidemia, blood stasis, tissue hypoxia –> all of these lead to organ failure
  3. Fibrinolysis and Antithrombotic mechanisms activate for anticoagulation to occur
  4. Platelets and coagulation factors are consumed and hemorrhaging will then begin
169
Q

What value of PLT indicates DIC

A

<100,000 cells per microliter

170
Q

What value of Fibrinogen indicates DIC

A

<150 mg/dL

171
Q

What value of PT indicates DIC

A

> 15 seconds (these are the Vitamin K factors)

172
Q

What value of PTT indicates DIC

A

> 60-80 seconds (these are the non Vit K factors)

173
Q

Other than PLT, Fibrinogen, PT, and PTT values, what else can diagnose DIC?

A

Fibrin Degradation Products and a D Dimer Test (both measure for fibrinolytic system waste)

174
Q

What value of Fibrin Degradation Products (FDP) indicates DIC

A

> 45 mcg/mL

175
Q

What is a positive value for a D Dimer Test, checking waste of the fibrinolytic system that breaks clots down, for DIC?

A

< 1:8 dilution

176
Q

Treatment for DIC

A
  1. Prompt treatment of underlying disorder (earlier is better)
  2. If there is active bleeding administer fresh frozen platelets, platelets, and packed RBCs
  3. (Controversial) Heparin Therapy if early enough
177
Q

Why is Heparin therapy for DIC controversial?

A

It requires a delicate balance between clotting factors and clot busting drugs

If used early in the course of DIC it may prevent micro clotting, but its not useful later on and could exacerbate problems

178
Q

Heparin therapy, in most cases, is administered with…

A

transfusion therapy

179
Q

Fibrinolysis

A

“Clot Busting System”

180
Q

Components of the Fibrinolytic System

A

Plasminogen

Plasmin

Urokinase

Tissue Plasminogen Activators (TPA)

181
Q

Plasminogen

A

Present in plasma

a plasmin precursor

acted upon by activators like urokinase present in tissues, endothelial cells, and granulocytes which detect presence of a clot

182
Q

Plasmin

A

Also called fibrinolysin

active enzyme (protease) that cleaves or lyse fibrin or fibrinogens to soluble fragments that in turn binds fibrin monomers preventing further polymerization (prevents the fibrin cement for the plug)

cleared by the liver

183
Q

Urokinase

A

Protein secreted by kidney cells that directly activate plasminogen into plasmin

can be given IV, 6 hours after an MI, and can be used to break down pulmonary emoboli

184
Q

Tissue Plasminogen Activators (TPA)

A

Compounds only active only in the presence of fibrin

Helps activate Plasminogen

Can be used to stop an embolic stroke and de clot a central line

185
Q

Which kind of Stroke can TPA be used for

A

Embolic Stroke

*NOT Hemorrhagic, that would make it worse

186
Q

How can TPA be useful with a central line?

A

If you are unable to draw up blood from the line that’s supped to get big pollutants out of the heart, you can use this to break up the fibrin sheathe at the end of the line and prevent having to put in a new surgical line

187
Q

Clotting occurs at the same time as de-clotting to allow

A

clotting localization

188
Q

What does the Fibrinolytic System do?

A

Breaks up existing clots

It lyses fibrin and causes inactivation of fibrinogen via factors

It also interferes with platelet aggregation and increases time needed to convert prothrombin to thrombin (inhibition of further clot formation)

Measure FDP to determine effectiveness

189
Q

Which factors does the fibrinolytic system impact?

A

Facto I

Factor V

Factor XIII

190
Q

Why does DVT need natural body healing rather than massage or heparin?

A

Those things can prevent further clots or dislodge the clot but cannot care for existing clots

the plasmin the body can break down existing clots though

191
Q

What value is often used instead of PT and PTT?

A

INR

192
Q

What is a normal INR value

A

1

193
Q

What value should INR be for mild clot prevention? Aggressive clot prevention?

A

mild - 2.5

aggressive - up to 4

*INR is 2-4x higher when trying to anticoagulate someone

194
Q

Warfarin

A

Generic name for Coumadin

Measure PT with this since it impacts VitK factors

More Vit K = decreased effectiveness

Need proper Vit K Amounts

195
Q

Lovenox

A

low weight form of heparin

Measure PTT since it affects non VitK dependent factors

people can go home with this form

196
Q

What is the antidote for Lovenox/Heparin?

A

Protamine Sulfate

197
Q

What is the antidote for Warfarin/Coumadin?

A

Vitamin K

198
Q

The goal of anticoagulation is ___

A

balance

N361: Pathophysiology II (20 decks)

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