IHO Week 6 > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

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1
Q

What are the hereditary bleeding disorders we’re studying?

A
  1. Von Willebrand Disease
  2. Hemophilia A and B
  3. Hereditary platelet disorders
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2
Q

What are the acquired bleeding disorders we’re studying?

A
  1. DIC
  2. ITP
  3. TTP/HUS
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3
Q

What are the two types of bleeding?

A
  1. Platelet bleeding

2. Factor bleeding

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4
Q

What should you know about Platelet bleeding?

A
  • Superficial (skin)
  • Petechiae
  • Patient doesn’t recall being hurt
  • Spontaneous
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5
Q

What should you know about Factor bleeding?

A
  • Deep (joints)
  • Big bleeds
  • Trauma
  • Patient recalls incident when they were hurt
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6
Q

What are Petechiae?

A

Little red dots on surface of skin

-Symptoms you should think about with platelet bleeding

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7
Q

What symptom should you think about with platelet bleeding?

A

Petechiae

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8
Q

What is purpura?

A

Coalescence of petechiae into a large, red area

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9
Q

What MUST you know about Von Willebrand Disease?

A
  • Most common hereditary bleeding disorder (almost 1/100)
  • Autosomal dominant
  • vW factor decreased (or abnormal)
  • Variable severity
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10
Q

What’s Von Willebrand factor?

A
  • Huge multimeric protein
  • Made by megs and endothelial cells
  • Glues platelets to endothelium
  • Carries factor VIII
  • Decreased or abnormal in vW disease
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11
Q

What are the 3 types of Von Willebrand Disease (EXAM!!)?

A 
Type I  (70%) - Decreased vWF
Type II (25%) - Many subtypes, abnormal vWF but right amount
Type III (5%) - No vWF
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12
Q

What does Factor VIII (carried by vWF) do?

A

Factor VIII - acts on IX to accelerate coagulation through intrinsic pathway

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13
Q

What are the symptoms of Von Willebrand Disease?

A
  • Mucosal bleeding in most patients
  • Deep joint bleeding in severe cases
  • Common symptom for woman is very heavy periods
  • Nosebleeds, easy bruising
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14
Q

What are the Lab Test results for vWD?

A
  • Bleeding time: prolonged
  • PTT: prolonged (“corrects” with mixing study)
  • INR: normal
  • vWF level decreased (normal in type 2)
  • Platelet aggregation studies abnormal
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15
Q

What platelet membrane protein binds vWF?

A

GP Ib

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16
Q

What is Ristocetin? How does it interact with vWF?

A

Ristocetin is a platelet aggregator. It works by stimulating platelets to express glycoprotein Ib factors. It normally makes platelets aggregate. If you don’t have any vWF to bind GP Ib Ristocetin is not going to cause platelet aggregation.

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17
Q

How do you treat Von Willebrand Disease?

A
  1. DDAVP (raises VIII and vWF levels)
  2. Cryoprecipitate (contains vWF and VIII)
  3. Factor VIII (this is the main problem in most cases - works for Type I but not very good for Type III)
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18
Q

How does Hemophilia A & B relate to royalty?

A

Queen had spontaneous mutation an passed that gene down to her children. Then her children married into the royal families and there were a lot of problems (usually hemophilia is an X-linked recessive disorder but in this case it was a mutation)

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19
Q

How do mothers pass down the hemophilia genes?

A

X-linked

  • Daughters become carriers
  • Sons are affected
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20
Q

How does an affected father pass down hemophilia genes?

A

He will only make his daughters carriers. It will not affect his sons, but most of these conditions only manifest in males.

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21
Q

What things MUST you know about Hemophilia A?

A
  • Most common factor deficiency
  • X-linked recessive in most cases (30% are random mutations)
  • Factor VIII level decreased
  • Variable amount of “factor” bleeding
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22
Q

What should you know about Hemophilia A and B?

A
  • Overall very uncommon

- Most common factor diseases

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23
Q

What are the symptoms of Hemophilia A?

A
  • Severity depends on amount of VIII
  • Typical “factor” bleeding
  • –Deep joint bleeding
  • –Prolonged bleeding after dental work
  • Rarely, mucosal hemorrhage
  • Can have hemophilic arthropathy of the knee/joint deformity
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24
Q

What are the Lab Test results in Hemophilia A?

A
  • INR, TT, platelet count, bleeding time: normal
  • PTT: prolonged (“corrects” with mixing study)
  • Factor VIII assays: abnormal
  • DNA studies: abnormal
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25
What is the treatment for Hemophilia A?
- DDAVP (Desmopressin) - Factor VIII - Patients will continually need more and more Factor VIII (you save this for when they really need it and don't give on a routine basis)
26
What things MUST you know about Hemophilia B?
- Factor IX level decreased (intrinsic pathway) - Much less common than hemophilia A - Same inheritance pattern - Same clinical and laboratory findings
27
What should you know about Other Factor Deficiencies?
- These are rare - XI deficiency: bleeding only after trauma - XIII deficiency: severe neonatal bleeding
28
What pathway does Hemophilia A affect?
Intrinsic pathway - affects Factor VIII
29
What are the four hereditary platelet disorders?
1. Bernard-Soulier Syndrome 2. Glanzmann Thrombasthenia 3. Gray Platelet Syndrome 4. Alpha Granule Deficiency
30
What is Bernard-Soulier Syndrome known for?
- Abnormal Ib (binds platelets to subendothelium, binds vWF) - Abnormal adhesion - Big platelets - look fuzzy, large, about size of RBC - Severe bleeding - can't adhere their platelets into the sub endothelium
31
What is Glanzmann Thrombasthenia known for?
- No IIb-IIIa - No aggregation - Severe bleeding
32
What is Gray Platelet Syndrome?
- No alpha granules - Big, empty platelets - Mild bleeding
33
What does Thrombasthenia mean?
Lazy platelets!
34
What is seen in Delta granule deficiency (KNOW!!)?
- No alpha granules | - Can be part of syndrome (e.g., Chediak-Higashi) - KNOW THIS!
35
What does the platelet aggregation study show in Bernard-Soulier?
These patients don't have normal Ib so it looks like vWD. | Ristopetin doesn't make platelets granulate
36
What does a platelet aggregation study show in Glanzmann?
Platelets don't aggregate at all - straight lines in all categories except Ristocetin -LAZY PLATELETS
37
What acquired bleeding disorder is the ONE TO KNOW?
DIC (disseminated intravascular coagulation)
38
What things MUST you know about Disseminated Intravascular Coagulation?
- Lots of underlying disorders - Something triggers coagulation, causing thrombosis - Platleets and factors get used up, causing bleeding - Microangiopathic (small vessels) hemolytic (cells getting busted open) anemia (red cells snag on strands, bust apart and get resealed)
39
What is the main problem in DIC (Disseminated Intravascular Coagulation)?
Main problem = widespread activation of coagulation (not platelet problem originally) - -> then you end up with micro thrombi (eventually you use up clotting factors and platelets and make FDPs) - -> In real life this all shows up at once, not in stages/progression
40
What are the stages of DIC?
1. Widespread activation of coagulation 2. Endothelial damage 3. Generalized platelet aggregation 4. Microthrombi in the circulation 5. This causes (1) Dec. clotting factors (2) Dec. platelets (3) Inc. FDPs
41
What symptoms can DIC cause?
- Bleeding (purpura, petechia) | - Amputation required
42
What are the two main causes of DIC?
1. Dumpers (dump procoagulant substances into the blood) | 2. Rippers (rip endothelium up)
43
What is associated with Dumpers (DIC)?
- Obstetric complications (amniotic fluid can get back up into the blood and cause DIC) - Adenocarcinoma (mucin in adenocarcinoma activates the cascade) - Acute promyelocytic leukemia
44
What is associated with Rippers (DIC)?
-Bacterial sepsis -Trauma -Burns -Vasculitis [these rip endothelium up]
45
What should you remember for sure with DIC? (EXAM!!!)
- Malignancy - OB complications - Sepsis - Trauma
46
What are the main symptoms of DIC?
- Insidious (usually not) or fulminant - Multi-system disease - Thrombosis and/or bleeding
47
What are the Lab Test Results in DIC?
ALL ABNORMAL INR, PTT, TT: prolonged FDPs: increased (doesn't really prove anything - but negative would rule DIC out) -Fibrinogen: decreased
48
What is a way to remember how severe DIC is?
Death is Coming
49
How do you treat DIC?
- Treat underlying disorder | - Support with blood products
50
What does ITP stand for?
Idiopathic Thrombocytopenic Purpura
51
What MUST you know about Idiopathic Thrombocytopenic Purpura?
- Antiplatelet antibodies - Acute vs. Chronia - Diagnosis of exclusion - Steroids or splenectomy
52
What is the pathogenesis of ITP?
- Autoantibodies to GP IIb-IIIa or Ib - Bind to platelets (yummy!) - Splenic macrophages eat platelets
53
What is associated with CHRONIC ITP?
- Adult women - Primary or secondary - Insidious: nosebleeds, easy bruising - Danger: bleeding into brain
54
What is associated with ACUTE ITP?
- Children - Abrupt; follows viral illness - Usually self-limiting - May become chronic
55
What are the results of lab tests in ITP?
- Signs of platelet destruction: - -Thrombocytopenia - -Normal/increased megakaryocytes - -Big platelets - INR/PTT normal - No specific diagnostic test for ITP
56
What are some other causes of Thrombocytopenia?
- Aplastic Anemia - Bone marrow replacement - Big spleen - Consumptive processes (DIC, TTP, HUS) - Drugs
57
What is thrombocytopenia?
Decrease in platelets!
58
What are two things you can see in a slide of ITP?
Thrombocytopenia and big platelets (not in every patient) | Increased bone marrow megakaryocytes (very large)
59
How do you treat ITP?
1. Glucocorticoids 2. Intravenous immunoglobulin 3. Splenectomy (doesn't cure but this is the site of destruction so you won't be destroying cells as much -- this a last resort treatment)
60
What are the acquired bleeding disorders?
1. DIC 2. ITP 3. TTP/HUS
61
Why are TTP and HUS different?
- They have different causes | - Different clinical symptoms
62
What are Thrombotic Microangiopathies?
- All have thrombi, thrombocytopenia, and MAHA - Include TTP and HUS - Can be hard to distinguish TTP from HUS - Something triggers platelet activation - Different from DIC!!
63
What MUST you know about Thrombotic Thrombocytopenia Purpora? (EXAM!)
- Pentad: MAHA, thrombocytopenia, fever, neurologic defects, renal failure - Deficiency of ADAMTS13 - Big vWF multimers trap platelets - Plasmapheresis or plasma infusions
64
What is the Pentad you must know about TTP?
MAHA (microangiopathic hemolytic anemia), thrombocytopenia, fever, neurologic defects, renal failure
65
What is the pathogenesis of TTP?
- Just-released vWF is unusually large (UL) - UL vWF causes platelet aggregation - ADAMTS13 cleaves UL vWF into less active bits! (ADAMTS13 is an enzyme that cleaves vWF) - TTP is due to ADAMTS13 deficiency
66
What does the Weibel Palade body secrete?
vWF
67
What 'nasty creature' do you see in TTP?
Von Willebrand Multimer of unusual size (MOUS)
68
What are the clinical findings in TTP (EXAM!!)?
- Hematuria, jaundice (MAHA) - Bleeding, bruising (thrombocytopenia) - Fever - Bizarre behavior (neurologic deficits) - due to little clots "showering" the brain - Decreased urine output (renal failure)
69
How do you treat acquired TTP vs. hereditary TTP?
Acquired TTP: Plasmapheresis (removing 'bad' plasma) | Hereditary TTP: Plasma infusions (giving patient plasma)
70
What do you have to remember about Epidemic Hemolytic Uremic Syndrome (EXAM!!)?
E. coli!!
71
What is HUS?
Hemolytic Uremic Syndrome (thrombosis in little vessels)
72
What MUST you know about Hemolytic Uremic Syndrome?
- MAHA and thrombocytopenia - Epidemic (E. coli) vs. non-epidemic - Toxin (or ?) damaged endothelium - Treat supportively
73
What is the pathogenesis of epidemic HUS?
- E. coli O157:H7 (raw hamburger) - Makes nasty toxin - Injures endothelial cells (this injury activates platelets)
74
What is the pathogenesis of non-epidemic HUS?
- Defect in complement factor H (protects our cells from bursting open) - Inherited or acquired - How does this activate platelets?
75
What are the clinical findings in Epidemic HUS?
- Children, elderly - (will have anuria - not urinating) Bloody diarrhea, then renal failure - Fatal in 5% of cases
76
What are the clinical findings in Non-epidemic HUS?
- Renal failure - Relapsing-remitting course - Fatal in 50% of cases
77
What ORGAN is associated with TTP (EXAM)?
BRAIN
78
What ORGAN is associated with HUS (EXAM)?
KIDNEY
79
What is the treatment of HUS?
- Supportive care - Dialysis (if anuria) - NOT antibiotics (may increase toxin release! - bust open bugs that are present
IHO Week 6
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