#25 - bleeding disorders Flashcards

1
Q

2 categories of bleeding disorders

A

1-platelet disorders

2-coagulation factor disorders

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2
Q

2 categories of bleeding disorders

A

1-platelet disorders

2-coagulation factor disorders

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3
Q

mucocutaneous bleeding, bruises/echchymoses, petechiae suggest which type of disorder?,

A

platelet defect

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4
Q

platelet defect usually leads to which type of bleeding?

A

bleeding, bruises/echchymoses, petechiae

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5
Q

hemarthrosis, hematuria, deep hematoma all suggest what type of bleeding disorder?

A

coagulation factor defect

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6
Q

bruising, mucocutaneous bleeding, and hemarthrosis (bleed into joint) suggest which type of disorder?

A

both types of bleeding = suggest von Willebrand disease

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7
Q

What is the normal level of platelets?

A

150,000 to 450,000

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8
Q

At what level of platelets will spontaneous (life threatening) bleeding occur?

A
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9
Q

prothrombin time (PT) tests which process of coagulation / factors?

A

initiation of coagulation .

factor 7

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10
Q

PTT tests which factors?

A

8 and 9

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11
Q

PT is prolonged by what?

A
  • liver disease
  • vit K deficiency
  • warfarin
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12
Q

PTT is prolonged by what?

A
  • hemophilia
  • heparin
  • coagulation inhibitors
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13
Q

mucocutaneous bleeding, bruises/echchymoses, petechiae suggest which type of disorder?,

A

platelet defect

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14
Q

platelet defect usually leads to which type of bleeding?

A

bleeding, bruises/echchymoses, petechiae

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15
Q

hemarthrosis, hematuria, deep hematoma all suggest what type of bleeding disorder?

A

coagulation factor defect

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16
Q

bruising, mucocutaneous bleeding, and hemarthrosis (bleed into joint) suggest which type of disorder?

A

both types of bleeding = suggest von Willebrand disease

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17
Q

Clinical Presentation of Thrombotic thrombocytopenic purpura

A
  • thrombocytopenia
  • renal dysfunction
  • fever
  • rash
  • petechiae in oral mucosa
  • 3+ schistocytes!!
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18
Q

At what level of platelets will spontaneous (life threatening) bleeding occur?

A
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19
Q

At what level of platelets will increased risk of bleeding occur?

A
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20
Q

prothrombin time (PT) tests which factor?

A

factor 7

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21
Q

PTT tests which factor?

22
Q

PT is prolonged by what?

A
  • liver disease
  • vit K deficiency
  • warfarin
23
Q

PTT is prolonged by what?

A
  • hemophilia (factor 8 and 9 deficiency),
  • coagulation factor inhibitors
  • heparin
24
Q

T/F Acquired (qualitative) platelet defects are common

A

True (congenital defects are rare)

25
what is idiopathic/immune thrombocytopenic purpura?
severe thrombocytopenia, usually
26
treatment for ITP (idiopathic/immune thrombocytopenic purpura)
- children - self limited - not needed | - adults - it is chronic, treatment needed. corticosteroids.
27
Schistocytes on the peripheral blood smear indicate
microangiopathic hemolysis
28
Clinical Presentation of Thrombotic thrombocytopenic purpura
- thrombocytopenia - renal dysfunction - fever - bleeding - 3+ schistocytes!!
29
Treatment of TTP - Thrombotic thrombocytopenic purpura
hematologic emergency!! | urgent plasma exchange
30
What causes TTP? (Thrombotic thrombocytopenic purpura)
Many cases caused by deficiency of vWF - cleaving protease (ADAMTS13). You end up with ultra-large multimers of vWF, which bind platelets too much, causing thrombosis and lowered platelet counts
31
labs in TTP (Thrombotic thrombocytopenic purpura)
- PT and APTT usually normal | - high LDH
32
What is the role of von WIllebrand factor?
- it facilitates platelet adhesion to injured endothelium - it binds and carries factor 8 in plasma (this is why it causes platelet and coag factor bleeding)
33
Treatment for hemophilia b
- factor 9 concentrate for everyone, all severities. | - antifibrinolytic agent for
34
complications of hemophilia treatment
- expensive | - pt's develop antibodies to the factors they are given.
35
3 common causes of acquired platelet defects
- Drugs (NSAIDs, clopidogrel) - uremia/ renal disease - liver failure
36
characteristics of von willebrand disease - clinical presentation
- lifelong bleeding - -prolonged PTT, which corrects with mixing. - prolonged PFA-100 - decreased levels of vWF and Factor 8
37
Who gets hemophilia A and B?
young boys - they are X linked recessive disorders
38
presentation of hemophilia
young boy | -prolonged PTT which corrects to normal with 1:1 mixing.
39
What levels of factor 8/9 define severe, moderate and mild?
severe = 5% only severe have spontaneous bleeds. Mild and moderate don't have many symptoms
40
clinical findings for hemophilia
- hemarthrosis, hematuria, hematoma - excessive surgical bleeding - arthropathy
41
Treatment for mild hemophilia A
desmopressin (releases vWF from endothelium, increasing factor 8)
42
Treatment for moderate/severe hemophilia A
factor 8 concentrate (only for moderate / severe cases) | -antifibrinolytic agent for bleeding
43
Treatment for hemophilia b
- factor 9 concentrate for everyone, all severities. | - antifibrinolytic agent for
44
complications of hemophilia treatment
- expensive | - pt's develop antibodies to the factors they are given.
45
characteristics of factor inhibitor diseases
don't correct with mixing. -result in low levels of a specific factor (eg if it's a factor 8 inhibitor, it will show low factor 8 on labs) - can be spontaneous, or as a result of hemophilia therapy. - severe and difficult to control bleeding.
46
characteristics of von willebrand disease - clinical presentation
--prolonged PTT, which corrects with mixing. -prolonged PFA-100 -
47
difference between type 1 and type 2 and type 3 von willebrand disease.
type 1 = quantitative deficiency of vWF type 2 - qualitative deficiency of vWF type 3 - complete absence of vWF
48
Treatment for mild von willebrand disease
desmopressin
49
Treatment for type 3 von willebrand disease (absence of vWF)
factor 8 / vWF concentrates
50
how do you differentiate TTP from DIC?
In TTP, PT and PTT are normal.
51
what is hemolytic uremic syndrome and what causes it?
HUS is a variant of TTP. It is the leading cause of renal failure in children worldwide. It is NOT caused by deficiency of ADAMTS13, it is caused by E coli shiga toxin.
52
What are the 4 most common causes of qualitative platelet defects?
- congenital - drugs - renal disease - liver disease