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Pathology - Hematology > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

activation of coagulation cascade takes place in association with…

A

phospholipids (platelet/cell surface)
Ca
Cofactors

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2
Q

Nearly all coagulation proteins are produced where_____

Exception?

A

in the liver

vW n the endothelial cells

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3
Q

Name the Vit K ones…

A

2, 7, 9, 10, C, S

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4
Q

Deficiencies of which factors are not associated with bleeding tendencies

A

12, prekalikrein, HMW kininogen

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5
Q

How is coagulation inhibited?

A
  1. Plasminogen-Plasmin system breaks down fibrin polymerization, cleavage to plasmin by activators
  2. ANTITHROMBINS
  3. PROTEIN C + S
  4. C1 ESTERASE INHIBITOR, ALPHA 2 MACROGLOBULIN
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6
Q

PROTEIN C AND S INHIBIT…

A

FACTOR 5 AND FACTOR 8

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7
Q

Antithrombins inhibit the activity of…

A

thrombin

9a, 10a, 11s, 12a

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8
Q

Platelet structural features tested as…

A

Platelet factor 3

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9
Q

Thrombin activated ______. So what?

A

FACTOR 13
This is a fibrin-stabilizing factor that allows for cross linking. This make a clot resistant to solubilization in 6M urea

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10
Q

Importance of plasminogen activators

A

They induce the formation of active plasmin, which degrades several proteins, but especially plasminogen on fibrin

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11
Q

What is EACA?

A

a competitive inhibitor of plasminogen activators

Decreases fibrinolysis

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12
Q

Name two common drugs used as plasminogen activators therapeutically

A

Urokinase

Streptokinase

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13
Q

How does Heparin work?

A

potentiates anti-thrombin III

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14
Q

How does Coumadin work?

A

Interferes with Vit K carboxylation

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15
Q

What is DDAVP?

A

desmopressin acetate –> increases factor 8 levels transiently

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16
Q

What do patients get in fresh frozen plasma

A

Coag factors, no platelets

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17
Q

Normal Bleeding time

A

2-7 mintues

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18
Q

Which clotting factor being low can cause a low bleeding time

A

fibrinogen

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19
Q

INR is equivalent to which test

A

PT

20
Q

Solubility in 6M urea is a test for…

A

Factor 8 stabilization

21
Q

Ristocetin test is used to test

A

Specific aggregation of platelets due to vWF

22
Q

Causes of non-thrombocytopenic purpuras?

A

Infections, Drug reactions, Scurvy, Ehlers Danlos

23
Q

Infections that cause non-thrombocytopenic purpuras

A

Septicemia
Meningococcemia
Severe Measles
Rickettsia

24
Q

Thrombocytopenia is a platelet count of…

A

under 100,000

25
Q

Six causes of thrombocytopenia

A
  1. Bone Marrow Disease
  2. Ineffective megakeryopoesis (folate/B12)
  3. Immune Destruction
  4. Mechanical
  5. Hypersplenism (sequestration)
  6. Dilutional (from massive transfusion)
26
Q

Cause of neonatal thrombocytopenia

A

PLA1 antigens can lead to destruction of fetal platelets by antibodies made in mother (like erythro. fetalis)

27
Q

What is ITP? Secondary causes?

A

Platelets are destroyed by anti-platelet antibodies

SLE, AIDS, post-viral, drug therapy complication

28
Q

Target antigens in ITP

A

IIb/IIIa and Ib/IX

29
Q

Major form of immune damage in ITP?

A

mostly via IgG

Phagocytosis follows

30
Q

Why do a splenectomy for ITP

A

Decrease antibody formation and sequestration

31
Q

Clinical features of ITP

A

Women of CB age
Bruising, excessive bleeding, epistaxis
Treat w/ steroids

32
Q

Pentad of symptoms of TTP

A

Fever, Thrombocytopenia, MAHA, Neurological deficits, renal failure

33
Q

TTP leads to widespread formation of….

A

Hyaline thrombi in microcirculation
Dense platelet aggregates + fibrin
Large multimers of vWF/complex leading to microvascular thrombosis

34
Q

How to distinguish HUS from TTP

A

Absence of neurological symptoms

Mostly a renal problem

35
Q

Inheritance for hemophillia A

A

X linked

36
Q

Inheritance for vW disease

A

autosomal dominant

37
Q

What is Leiden Factor V

A

Arg–>Glu in Factor 5 makes resistant to protein C

Causes Deep vein thromboses

38
Q

Other than Leiden Factor V, other important hereditary hypercoagu. disorders

A

Prothrombin Deficiency
Hyperhomocysteinurea
Protein C or S Deficiency

39
Q

What is Heparin induced thrombocytopenia syndrome

A

Unfrax. heparin induces formation of antibodies to complexes of heparin and PF4

40
Q

What is anti-phospholipid syndrome

A

Make anti-phospholipid antibodies in SLE
Cardiac valvular vegitations, repeated miscarriages
False positive for Syphilis

41
Q

medication classically associated with hypercoagubility

A

oral contraceptives

42
Q

What is ticlopidine

A

ADP antagonist

43
Q

What is abciximab

A

monoclonal Ab to GpIIb-IIIa

44
Q

How does TTP present differently than DIC

A

In DIC you use up all the coagulation factors

45
Q

You need Factor ___ or the clot will stabilize in 6M urea

A

13

Pathology - Hematology (9 decks)

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