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SBL > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

What are the 4 steps to normal hemostasis (clot formation)?

A
  1. Vasoconstriction
  2. Platelet plug formation
  3. Coagulation cascade to stabilize clot
  4. Limit fibrin clot
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2
Q

If there is a primary hemostasis disorder that causes excessive bleeding, how is it likely inherited?

A 
Autosomal Dominant
(primary = platelet plug formation)
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3
Q

If there is a secondary hemostasis disorder that causes excessive bleeding, where are the bleeds usually located?

A

Joints - hemarthroses

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4
Q

4 types of bleeding disorders?

A
  • Vessel wall abnormalities
  • Reduced platelet number
  • Reduced platelet function
  • Clotting factor abnormalities
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5
Q

PT measures?

A

Extrinsic pathway (tissue factor + FVII)

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6
Q

PTT measures?

A

Intrinsic pathway

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7
Q

What is an example of a vessel wall abnormality that leads to excessive bleeding in the periorbital region?

A

Perivascular amyloid in myeloma

= periorbital bruising (raccoon eyes)

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8
Q

< 150K platelets

A

Thrombocytopenia

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9
Q

Symptoms for thrombocytopenia usually manifest at what number of platelets?

A

< 50K

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10
Q

Dangerous spontaneous bleeds with thrombocytopenia can occur when the number of platelets is below?

A

< 20K

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11
Q

What are 3 immune destruction disorders of platelets?

A
  1. HIV-associated thrombocytopenia
  2. Drug-induced thrombocytopenia
  3. Immune Thrombocytopenic Purpura (ITP)
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12
Q

HIV-associated thrombocytopenia suppresses bone marrow by?

A

Infecting hematopoietic stem cells

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13
Q

HIV-associated thrombocytopenia will produce _____ against platelets

A

Autoantibodies

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14
Q

Drug-induced thrombocytopenia via Myelosuppression occurs from?

A

Chemotherapy, Chloramphenicol and others

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15
Q

Drug-induced immune thrombocytopenia occurs from?

A

Quinidine, Vancomycin, Heparin

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16
Q

Heparin induced immune thrombocytopenia involves what cascade of events?

A

PF4 - Heparin complex activates IgG

  • IgG binds to the Fc receptor and activates platelets
  • Platelets are degraded by IgG
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17
Q

What happens with Immune Thrombocytopenic Purpura (ITP)?

A

Autoantibodies against platelets bind to platelets

- Macrophages destroy platelets

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18
Q

Acute form of Immune Thrombocytopenic Pupura occurs in?

A

Kids

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19
Q

Chronic form of immune thrombocytopenic purpura occurs in?

A

Young adult women

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20
Q

What are the symptoms seen with Immune Thrombocytopenic Purpura?

A

Petechiae, purpura, thrombocytopenia, platelet antibodies and increased megakaryocytes in marrow

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21
Q

Increased Megakaryocytes in the bone marrow could indicate?

A

Immune Thrombocytopenic Purpura

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22
Q

Treatment for Immune Thrombocytopenic Purpura?

A

Corticosteroids
IVIg
CD20 - rituximab

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23
Q

What are 2 Non-immune destruction disorders of platelets?

A

Thrombotic Thrombocytopenia Purpura (TTP)

Hemolytic Uremic Syndrome

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24
Q

Normally, what metalloproteinase converts multimers of vWF to smaller multimers?

A

ADAMTS13

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25
Q

In Thrombotic Thrombocytopenic Purpura, what is missing or inhibited?

A

ADAMTS13

26
Q

Results of Thrombotic Thrombocytopenic Purpura missing ADAMTS13?

A

Formation of ultra-large multimers of vWF that initiate aggregation and thrombosis

27
Q

What is the treatment for Thrombotic Thrombocytopenic Purpura?

A

Plasma exchange therapy to take out autoantibodies for ADAMTS13 and multimers and give ADAMTS13

28
Q

What are the 5 main symptoms of Thrombotic Thrombocytopenic Purpura?

A 
Fever
Thrombocytopenia
MAHA - Microangiopathic Hemolytic Anemia
Renal failure
Neurologic Defects
29
Q

What toxin is associated with Hemolytic Uremic Syndrome?

A

Shiga-like toxin elaborated by E.coli 0157 H7

30
Q

Patients with Hemolytic Uremic Syndrome present first with?

A

Bloody diarrhea

31
Q

Patients with Hemolytic Uremic Syndrome present with what 3 main symptoms?

A

MAHA
Thrombocytopenia
Renal failure

32
Q

What is MAHA?

A

Microangiopathic Hemolytic Anemia

= Platelet aggregation causes passing RBCs to shear and create schistocytes

33
Q

What 2 platelet destruction disorders involve MAHA?

A

Thrombotic Thombocytopenic Purpura
Hemolytic Uremic Syndrome
= when platelet aggregation causes passing RBCs to shear and create schistocytes

34
Q

What are 3 inherited disorders due to defective platelet function?

A

Bernard - soulier
Glanzmann Thrombasthenia
Storage pool disorders

35
Q

What are 2 acquired disorders due to defective platelet function?

A

Aspirin use

Uremia

36
Q

No adhesion, lack of GP1b

A

Bernard - Soulier

37
Q

No aggregation, lack of GP2b3a

A

Glanzmann Thrombasthenia

38
Q

No release of dense granules

A

Storage pool disorders

39
Q

No release of alpha granules

A

Grey platelet syndrome

40
Q

Platelet vs. coagulation disorders - depth of bleed

A 
Platelet = superficial mucosal bleeding
Coagulation = deep soft tissue bleeding
41
Q

Platelet vs. coagulation disorders - petechiae?

A 
Platelet = petechiae present
Coagulation = Minimal petechiae
42
Q

Platelet vs. coagulation disorders - bruise size

A 
Platelet = small, superficial bruises
Coagulation = Large, deep bruises
43
Q

Platelet vs. coagulation disorders - hemarthroses?

A 
Platelet = rare hemarthroses
Coagulation = common hemarthroses
44
Q

Platelet vs. coagulation disorders - time to bleed?

A 
Platelet = bleeding after minor cuts
Coagulation = delayed bleeding after cuts
45
Q

Why is it important for circulating vWF to join factor VIII?

A

It takes VIII to the site of the platelet plug so that factor VIII can activate the coagulation cascade and further stabilize the clot

46
Q

vWF disease type 1

A

Decreased secretion of vWF

47
Q

vWF disease type 1c

A

Increased clearance of vWF

48
Q

vWF disease type 3

A

SEVERE decreased secretion of vWF

49
Q

vWF disease type 2

A

lack of appropriate interaction with ligands

50
Q

vWF disease type 2a

A

Lack of multimer assembly

51
Q

Hemophilia A involves what coagulation factor?

A

VIII

52
Q

Hemophilia B involves what coagulation factor?

A

IX

53
Q

How are both Hemophilia A and B inherited?

A

X-linked recessive

54
Q

Overall summary of Disseminated Intravascular Coagulation?

A

Coagulation activates
Thrombi form
Everything gets used up
Bleed occurs

55
Q

With DIC, tissue injury, sepsis and conception products cause what to be release?

A

Tissue factor

56
Q

The release of tissue factor causes what with DIC?

A

Widespread thrombosis

57
Q

With DIC, widespread thrombosis causes what main things?

A
  1. Consumption of platelets and clotting factors

2. Proteolysis of clotting factors

58
Q

Consumption of platelets and clotting factors with DIC will cause ____ platelets and _____ PT/PTT

A

Decreased platelets

Increased PT/PTT

59
Q

Proteolysis of clotting factors with DIC will cause increased?

A

D-dimer

60
Q

What is the end result of DIC?

A

BLEEDING

SBL (48 decks)

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