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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
What characterizes Gray Platelet Syndrome?
* No alpha granules * can see with naked eye * Big, empty platelets * can see absence of granules with naked eye * Mild bleeding
26
What characterizes Delta Granule Deficiency?
* No delta granules * can't see with naked eye, need to look under microscope * Can be part of Chediak-Higashi syndrome
27
What characterizes Disseminated Intravascular Coagulation?
* 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
What are the causes of DIC?
* 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
What are the main four things that cause DIC?
1. Malignancy 2. OB complications 3. Sepsis 4. Trauma
30
What are the general symptoms of DIC?
* Insidious or fulminant * usually pt is sick and then DIC strikes * Multi-system disease * Thrombosis and/or bleeding
31
What are the laboratory test findings in DIC?
* INR, PTT, TT prolonged * not a problem with platelets * FDPs: increased * not super helpful * Fibrinogen: decreased
32
What is the treatment for DIC?
* Treat underlying disorder * Support with blood products
33
What characterizes Idiopathic Thrombocytopenic Purpura?
* Antiplatelet antibodies * Acute vs. chronic * Diagnosis of exclusion * Steroids or splenectomy
34
What is the pathogenesis of ITP?
* Autoantibodies to GP IIb-IIIa or Ib on platelets * Binds to platelets * prevents them from working * Splenic macrophages eat platelets
35
What characterizes the chronic form of ITP?
* Chronic: * Adult women * Primary or secondary * Insidious: nosebleeds, easy bruising * Danger: bleeding into brain
36
What characterizes the acute form of ITP?
* Children * Abrupt, follows viral illness * Usually self-limiting * May become chronic
37
What are the laboratory test findings in ITP?
* Signs of platelet destruction: * thrombocytopenia * normal/increased megakaryocytes * big platelets * INR/PTT normal * No specific diagnostic test for ITP
38
What are some of the other causes of Thrombocytopenia?
* Aplastic anemia * Bone marrow replacement * Big spleen * Consumptive processes (DIC, TTP, HUS) * Drugs
39
What is the treatment for ITP?
* Glucocorticoids * Intravenous immunoglobulin * Splenectomy
40
What are the similarities in all Thrombotic Microangiopathies?
* 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
What characterizes Thrombotic Thrombocytopenic Purpura?
* 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
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 pieces * enzyme that cleaves vWF * TTP is due to ADAMTS13 deficiency
43
What are the clinical findings in TTP?
* 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
What is the treatment of TTP?
* Acquired TTP: plasmapheresis * Hereditary TTP: plasma infusions
45
What characterizes Hemolytic Uremic Syndrome?
* MAHA and thrombocytopenia * Epidemic (E.coli) vs. non-epidemic * Toxin released by E.coli damages endothelium * Treat supportively
46
What is the pathogenesis of Epidemic HUS?
* E.coli O157:H7 (raw hamburger) * Makes nasty toxin * Injures endothelial cells * activates platelets
47
What is the pathogenesis of Non-epidemic HUS?
* 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
What are the clinical findings in Epidemic HUS?
* Children, elderly * Bloody diarrhea, then renal failure * **thrombi in kidneys** * Fatal in 5% of cases
49
What are the clinical findings in Non-Epidemic HUS?
* Renal failure * Relapsing-remitting course * Fatal in 50% of cases
50
What is the treatment of Epidemic HUS?
* 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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