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MOHD Keystone Exam 2 > #25 - bleeding disorders > Flashcards

#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?

A

8 and 9

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
Q

what is idiopathic/immune thrombocytopenic purpura?

A

severe thrombocytopenia, usually

26
Q

treatment for ITP (idiopathic/immune thrombocytopenic purpura)

A
  • children - self limited - not needed

- adults - it is chronic, treatment needed. corticosteroids.

27
Q

Schistocytes on the peripheral blood smear indicate

A

microangiopathic hemolysis

28
Q

Clinical Presentation of Thrombotic thrombocytopenic purpura

A
  • thrombocytopenia
  • renal dysfunction
  • fever
  • bleeding
  • 3+ schistocytes!!
29
Q

Treatment of TTP - Thrombotic thrombocytopenic purpura

A

hematologic emergency!!

urgent plasma exchange

30
Q

What causes TTP? (Thrombotic thrombocytopenic purpura)

A

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
Q

labs in TTP (Thrombotic thrombocytopenic purpura)

A
  • PT and APTT usually normal

- high LDH

32
Q

What is the role of von WIllebrand factor?

A
  • 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
Q

Treatment for hemophilia b

A
  • factor 9 concentrate for everyone, all severities.

- antifibrinolytic agent for

34
Q

complications of hemophilia treatment

A
  • expensive

- pt’s develop antibodies to the factors they are given.

35
Q

3 common causes of acquired platelet defects

A
  • Drugs (NSAIDs, clopidogrel)
  • uremia/ renal disease
  • liver failure
36
Q

characteristics of von willebrand disease - clinical presentation

A
  • lifelong bleeding
  • -prolonged PTT, which corrects with mixing.
  • prolonged PFA-100
  • decreased levels of vWF and Factor 8
37
Q

Who gets hemophilia A and B?

A

young boys - they are X linked recessive disorders

38
Q

presentation of hemophilia

A

young boy

-prolonged PTT which corrects to normal with 1:1 mixing.

39
Q

What levels of factor 8/9 define severe, moderate and mild?

A

severe = 5%

only severe have spontaneous bleeds. Mild and moderate don’t have many symptoms

40
Q

clinical findings for hemophilia

A
  • hemarthrosis, hematuria, hematoma
  • excessive surgical bleeding
  • arthropathy
41
Q

Treatment for mild hemophilia A

A

desmopressin (releases vWF from endothelium, increasing factor 8)

42
Q

Treatment for moderate/severe hemophilia A

A

factor 8 concentrate (only for moderate / severe cases)

-antifibrinolytic agent for bleeding

43
Q

Treatment for hemophilia b

A
  • factor 9 concentrate for everyone, all severities.

- antifibrinolytic agent for

44
Q

complications of hemophilia treatment

A
  • expensive

- pt’s develop antibodies to the factors they are given.

45
Q

characteristics of factor inhibitor diseases

A

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
Q

characteristics of von willebrand disease - clinical presentation

A

–prolonged PTT, which corrects with mixing.
-prolonged PFA-100
-

47
Q

difference between type 1 and type 2 and type 3 von willebrand disease.

A

type 1 = quantitative deficiency of vWF
type 2 - qualitative deficiency of vWF
type 3 - complete absence of vWF

48
Q

Treatment for mild von willebrand disease

A

desmopressin

49
Q

Treatment for type 3 von willebrand disease (absence of vWF)

A

factor 8 / vWF concentrates

50
Q

how do you differentiate TTP from DIC?

A

In TTP, PT and PTT are normal.

51
Q

what is hemolytic uremic syndrome and what causes it?

A

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
Q

What are the 4 most common causes of qualitative platelet defects?

A
  • congenital
  • drugs
  • renal disease
  • liver disease

MOHD Keystone Exam 2 (14 decks)

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