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IHO Week 6 - WLB > Bleeding Disorders - Krafts > Flashcards

Bleeding Disorders - Krafts Flashcards

1
Q

What are the three hereditary bleeding disorders that we need to know?

A
  1. von Willebrand disease
  2. Hemophilia A and B
  3. Hereditary platelet disorders
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2
Q

What are the three acquired bleeding disorders that we need to know?

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

What is the difference between platelet bleeding and factor bleeding?

A
  • Platelet bleeding
    • spontaneous
    • superficial (skin & mucous membranes)
      • dental procedures
      • heavy menses
    • petechiae
  • Factor bleeding
    • preceded by trauma
    • deep (muscles and joint spaces)
    • big bleeds
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4
Q

What is the difference between petechiae and purpura?

A
  • Petechiae
    • small red spots
  • Purpura
    • coalescence of petechiae
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5
Q

What characterizes Von Willebrand Disease?

A
  • Most common hereditary bleeding disorder
  • Autosomal dominant
  • vW factor decreased (or abnormal)
  • Variable severity
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6
Q

What is von Willebrand Factor?

A
  • Huge multimeric protein
  • Made by megakaryocytes and endothelial cells
  • Glues platelets down to subendothelium
    • ​attaches to collagen proteins
  • Carries factor VIII (cofactor that works with IX)
  • Decreased or abnormal in vW disease
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7
Q

What are the three types of Von Willebrand Disease?

A
  • Type I
    • decreased vWF
    • 70% of cases
  • Type 2
    • abnormal vWF (doesn’t work properly)
    • 25% of cases
  • Type 3
    • no vWF
    • 5% of cases
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8
Q

What are the symptoms of Von Willebrand Disease?

A
  • Mucosal bleeding in most patients
  • Deep joint bleeding in severe cases
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9
Q

What are the labratory test findings in Von Willebrand Disease?

A
  • Bleeding time: prolonged
    • platelets cannot adhere to subendothelium
  • PTT: prolonged
    • measures intrinsic pathway (no VIII)
    • (“corrects” with mixing study)
  • INR: normal
    • measures extrinsic pathway
  • vWF level decreased (normal in type 2)
  • Platelet aggregation studies abnormal
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10
Q

What membrane glycoprotein binds vWF?

A

GP Ib

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11
Q

When diagnosing Von Willebrand Disease, what is the result when Ristocetin is added to serum in platelet aggregation studies?

A

Nothing, flat line

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12
Q

What is the treatment for Von Willebrand Disease?

A
  • DDAVP
    • raises VIII and vWF levels
    • only works if pt is able to make factors (Type 1)
  • Cryoprecipitate
    • contains vWF and VIII
    • try not to give because of potential for rejections (immune antibodies)
  • Factor VIII
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13
Q

What is the incidence of Von Willebrand Disease?

A

1 in 100

(relatively common, but most people do not know they have it)

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14
Q

What characterizes Hemophilia A?

A
  • Most common factor deficiency
  • X-linked recessive in most cases
    • hereditary cases manifest mostly in males
    • 30% are random mutations
  • Factor VIII level decreased
  • Variable amount of “factor” bleeding
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15
Q

What are the symptoms of Hemophilia A?

A
  • Severity depends on amount of VIII
  • Typical “factor” bleeding
    • deep joint bleeding
    • usually preceding trauma
    • prolonged bleeding after dental work
  • Rarely, mucosal hemorrhage
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16
Q

What is hemophilic arthropathy?

A
  • Bleeding into the joint cause malformation/erosion/deformity
    • bring in macrophages to get rid of blood
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17
Q

What are laboratory test findings in Hemophilia A?

A
  • INR, TT, platelet count, bleeding time: normal
    • can perform initial platelet plug okay
    • so they stop bleeding at normal rate
    • but unable to form fibrin plug, so rebleed
  • PTT: prolonged
    • measures intrinsic pathway
    • (“corrects” with mixing study)
  • Factor VIII assays: abnormal
  • DNA studies: abnormal
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18
Q

What is the treatment of Hemophilia A?

A
  • DDAVP
    • will need additional supplementation with blood product or
  • Factor VIII
    • pt can make antibodies against it
    • will need larger amounts every time
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19
Q

What characterizes Hemophilia B?

A
  • Factor IX level decreased
  • Much less common than hemophilia A
  • Same inheritance pattern
  • Same clinical and laboratory findings
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20
Q

What happens in XI deficiency?

A

bleeding only after trauma

(begining of intrinsic pathway)

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21
Q

What happens in XIII deficiency?

A

Severe neonatal bleeding

(unable to cross-link fibrin)

22
Q

What are the four Hereditary platelet disorders that we need to know?

A
  • Bernard-Soulier Syndrome
  • Glanzmann Thrombasthenia
  • Gray Platelet Syndrome
  • Delta Granule Deficiency
23
Q

What characterizes Bernard-Soulier Syndrome?

A
  • Abnormal GP Ib
  • Abnormal adhesion of platelets
  • Big platelets
  • Severe bleeding
24
Q

What characterizes Glanzmann Thrombasthenia?

A
  • No GP IIb-IIIa
    • can adhere to subendothelium
  • No aggregation
  • Severe bleeding
25
Q

What characterizes Gray Platelet Syndrome?

A
  • No alpha granules
    • can see with naked eye
  • Big, empty platelets
    • can see absence of granules with naked eye
  • Mild bleeding
26
Q

What characterizes Delta Granule Deficiency?

A
  • No delta granules
    • can’t see with naked eye, need to look under microscope
  • Can be part of Chediak-Higashi syndrome
27
Q

What characterizes Disseminated Intravascular Coagulation?

A
  • Lots of underlying disorders
    • more of a symptom
  • Something triggers coagulation cascade
    • causes thrombosis
    • main problem
  • Platelets and factors get used up
    • causes bleeding
  • Microangiopathic hemolytic anemia
    • fibrin strands bust apart RBCs
28
Q

What are the causes of DIC?

A
  • Dumpers (dump pro-coagulation factors into blood)
    • obstetric complications
      • amniotic fluid backwashes into blood
    • adenocarcinoma (initiates coag cascade)
    • acute promyelocytic leukemia
  • Rippers (anything that damages endothelium)
    • bacterial sepsis
    • trauma
    • burns
    • vasculitis
29
Q

What are the main four things that cause DIC?

A
  1. Malignancy
  2. OB complications
  3. Sepsis
  4. Trauma
30
Q

What are the general symptoms of DIC?

A
  • Insidious or fulminant
    • usually pt is sick and then DIC strikes
  • Multi-system disease
  • Thrombosis and/or bleeding
31
Q

What are the laboratory test findings in DIC?

A
  • INR, PTT, TT prolonged
    • not a problem with platelets
  • FDPs: increased
    • not super helpful
  • Fibrinogen: decreased
32
Q

What is the treatment for DIC?

A
  • Treat underlying disorder
  • Support with blood products
33
Q

What characterizes Idiopathic Thrombocytopenic Purpura?

A
  • Antiplatelet antibodies
  • Acute vs. chronic
  • Diagnosis of exclusion
  • Steroids or splenectomy
34
Q

What is the pathogenesis of ITP?

A
  • Autoantibodies to GP IIb-IIIa or Ib on platelets
  • Binds to platelets
    • prevents them from working
  • Splenic macrophages eat platelets
35
Q

What characterizes the chronic form of ITP?

A
  • Chronic:
    • Adult women
    • Primary or secondary
    • Insidious: nosebleeds, easy bruising
    • Danger: bleeding into brain
36
Q

What characterizes the acute form of ITP?

A
  • Children
  • Abrupt, follows viral illness
  • Usually self-limiting
  • May become chronic
37
Q

What are the laboratory test findings in ITP?

A
  • Signs of platelet destruction:
    • thrombocytopenia
    • normal/increased megakaryocytes
    • big platelets
  • INR/PTT normal
  • No specific diagnostic test for ITP
38
Q

What are some of the other causes of Thrombocytopenia?

A
  • Aplastic anemia
  • Bone marrow replacement
  • Big spleen
  • Consumptive processes (DIC, TTP, HUS)
  • Drugs
39
Q

What is the treatment for ITP?

A
  • Glucocorticoids
  • Intravenous immunoglobulin
  • Splenectomy
40
Q

What are the similarities in all Thrombotic Microangiopathies?

A
  • All have thrombi, thrombocytopenia, and MAHA
  • Include TTP and HUS
  • Can be hard to distinguish TTP from HUS
    • coags are normal
  • Something triggers platelet activation
    • ​clots are formed from platelets
  • Different from DIC!
41
Q

What characterizes Thrombotic Thrombocytopenic Purpura?

A
  • Sx Pentad:
    • MAHA
    • thrombocytopenia
    • fever
    • neurologic defects
    • renal failure
  • Deficiency of ADAMTS13
  • Big vWF multimers trap platelets
    • vWF doesn’t get chopped up properly
  • Tx: Plasmapheresis or plasma infusions
42
Q

What is the pathogenesis of TTP?

A
  • Just-released vWF is unusually large (UL)
  • UL vWF causes platelet aggregation
  • ADAMTS13 cleaves UL vWF into less active pieces
    • enzyme that cleaves vWF
  • TTP is due to ADAMTS13 deficiency
43
Q

What are the clinical findings in TTP?

A
  • Hematuria, jaundice
    • MAHA (microangiopathic hemolytic anemia)
  • Bleeding, bruising
    • thrombocytopenia
  • Fever
    • probably a cytokine thing
  • Bizarre behavior
    • neurologic deficits
    • little thrombi shower the brain
  • Decreased urine output
    • due to renal failure
    • little thrombi in the kidneys
44
Q

What is the treatment of TTP?

A
  • Acquired TTP: plasmapheresis
  • Hereditary TTP: plasma infusions
45
Q

What characterizes Hemolytic Uremic Syndrome?

A
  • MAHA and thrombocytopenia
  • Epidemic (E.coli) vs. non-epidemic
  • Toxin released by E.coli damages endothelium
  • Treat supportively
46
Q

What is the pathogenesis of Epidemic HUS?

A
  • E.coli O157:H7 (raw hamburger)
  • Makes nasty toxin
  • Injures endothelial cells
    • activates platelets
47
Q

What is the pathogenesis of Non-epidemic HUS?

A
  • Defect in complement factor H
    • protects our cells from complement busting cells open
    • activates platelets
  • Inherited or acquired
    • not very common
  • How does this activate platelets?
48
Q

What are the clinical findings in Epidemic HUS?

A
  • Children, elderly
  • Bloody diarrhea, then renal failure
    • thrombi in kidneys
  • Fatal in 5% of cases
49
Q

What are the clinical findings in Non-Epidemic HUS?

A
  • Renal failure
  • Relapsing-remitting course
  • Fatal in 50% of cases
50
Q

What is the treatment of Epidemic HUS?

A
  • Supportive care
  • Dialysis if not making urine
  • NOT antibiotics
    • may kill bacteria and increase toxin release!

IHO Week 6 - WLB (8 decks)

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