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🩸Hema🎀 > 7.1: Disorders of the Coagulation System > Flashcards

7.1: Disorders of the Coagulation System Flashcards

1
Q

is defined as any single or multiple coagulation factor or platelet deficiency

A

Coagulopathy

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2
Q

Accounts for most instances of fatal hemorrhage

A

Trauma-Induced Coagulopathy

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3
Q

Is triggered by the combination of injury-related acute inflammation, hypothermia, acidosis, and hypoperfusion (elements of systemic shock)

A

Trauma-Induced Coagulopathy

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4
Q

Resembles the pathophysiology of TTP

A

Trauma-Induced Coagulopathy

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5
Q

Is defined as:
- Blood loss exceeding total blood volume within 24 hours
- Loss of 50% blood volume within a 3 hour period
- Blood loss exceeding 150 mL/min
- Blood loss that necessitates plasma and platelet transfusions

A

Massive Hemorrhage

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6
Q

is the key TIC management component

A

Plasma

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7
Q

Liver Disease Coagulopathy:

are a complication of chronic alcohol cirrhosis

A

Esophageal varices

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8
Q

Liver Disease Coagulopathy:

occurs in liver disease associated thrombocytopenia

A

Mucocutaneous bleeding

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9
Q

Liver Disease Coagulopathy:

is the consequence of procoagulant dysfunction and deficiency

A

Anatomic bleeding

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10
Q

serves as the sensitive early marker in Liver Disease Coagulopathy: Procoagulant deficiency

A

Factor VII

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11
Q

is a more specific marker of liver disease than deficient factors II, VII, IX, and X (used in conjunction with the factor VII assay

A

Factor V

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12
Q

fibrinogen is coated with excessive sialic acid (in moderately to severely diseased liver)

A

Dysfibrinogenemia

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13
Q

fibrinogen may fall to this level in end-stage liver disease

A

<100 mg/dL

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14
Q

Treatment to Resolve Liver Disease-Related Hemorrhage

A
  1. Oral or intravenous vitamin K therapy
  2. Plasma transfusion
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15
Q

Is often associated with platelet dysfunction and mild to moderate mucocutaneous bleeding (anemia and thrombocytopenia)

A

Chronic Renal Failure and Hemorrhage

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16
Q

Fibrin deposits in renal microvasculature reduce glomerular function

A

Chronic Renal Failure and Hemorrhage

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17
Q

Guanidinosuccinic acid or phenolic compounds coat the platelets

A

Chronic Renal Failure and Hemorrhage

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18
Q

What have been detected in urine in Nephrotic Syndrome and Hemorrhage

A

Clotting factors II, VII, IX, X, XII, antithrombin, and protein C

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19
Q

disrupts the vitamin K epoxide reductase and vitamin K quinone reductase reactions

A

Warfarin (Coumadin)

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20
Q

the most common acquired autoantibodies

A

Autoanti-factor VIII

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21
Q

Factor Inhibitors other than Autoanti-factor VIII:

develop aslupus anticoagulant

A

Antiprothrombin antibodies

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22
Q

Factor Inhibitors other than Autoanti-factor VIII:

documented in patients receiving isoniazid treatment for tuberculosis

A

Autoanti-factor XIII

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23
Q

Factor Inhibitors other than Autoanti-factor VIII:

may arise spontaneously in autoimmune disorders and after exposure to bovine thrombin in fibrin glue

A

Autoantibodies to factor V

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24
Q

Factor Inhibitors other than Autoanti-factor VIII:

in amyloidosis

A

Autoanti-factor X

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25
Q

Leads to decreased platelet adhesion to injure vessel walls

A

von Willebrand Disease

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26
Q

vWF gene:

supports the receptor site for collagen and GP Ib/IX/V

A

Domain A

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27
Q

vWF gene:

provides a site that binds GP IIb/IIIA

A

Domain C

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28
Q

vWF gene:

provides the carrier site for factor VIII

29
Q

Quantitative vWF deficiency

A

Type 1 von Willebrand Disease

30
Q

Qualitative vWF deficiency

A

Type 2 von Willebrand Disease

31
Q

Type 2 von Willebrand Disease

Arises from an autosomal dominant point mutations in A2 and D1 structural domains of the vWF molecule

A

Subtype 2A

32
Q

Type 2 von Willebrand Disease

vWF is susceptible to ADAMTS-13

A

Subtype 2A

33
Q

Type 2 von Willebrand Disease

Mutations within the A1 domain

A

Subtype 2B

34
Q

Type 2 von Willebrand Disease

Raised affinity to GP Ib/IX/IV

A

Subtype 2B

35
Q

Type 2 von Willebrand Disease

“Gain-of-function” mutation

A

Subtype 2B

36
Q

Type 2 von Willebrand Disease

HMW-VWF multimers spontaneously bind to resting platelet

A

Subtype 2B

37
Q

Type 2 von Willebrand Disease

a platelet mutation that raises GP Ib affinity for normal
HMW-VWF multimers

A

Subtype 2B, Platelet-type vWD (PT-VWD) or pseudo-vWD

38
Q

Type 2 von Willebrand Disease

Possess poor platelet receptor binding despite generating a normal multimeric distribution pattern in electrophoresis

A

Subtype 2M

39
Q

Type 2 von Willebrand Disease

Missence mutation in the D9 domain impairs the protein’s factor VIII binding site

A

Subtype 2N

40
Q

Type 2 von Willebrand Disease

Factor VIII deficiency despite a normal vWF antigen concentration assay result, normal vWF activity, and a normal multimeric pattern

A

Subtype 2N

41
Q

Type 2 von Willebrand Disease

Also known as Autosomal Hemophilia

A

Subtype 2N

42
Q

Subtype 2N is also known as

A

Normandy Variant

43
Q

“Null allele” vWF gene translation or deletion mutations

A

Type 3 von Willebrand Disease

44
Q

Is the most rare form of vWD

A

Type 3 von Willebrand Disease

45
Q

Partial quantitative deficiency of vWF

46
Q

Qualitative deficiency of vWF

47
Q

Decreased platelet-dependent vWF function and selective deficiency of high molecular weight multimers

48
Q

Increased affinity to platelet glycoprotein1B

49
Q

Decreased platelet dependent vWF function with high molecular weight multimers present

50
Q

Markedly decreased binding of factor VIII to vWF

51
Q

Complete deficiency of vWF

52
Q

Standard VWD test panel

A

A. VWF:Ag (quantitative)
B. VWF:RCo assay
C. Factor VIII assay

53
Q

Standard VWD test panel:

Most prominent member of the primary VWD laboratory profile

A

VWF:Ag (quantitative)

54
Q

Standard VWD test panel:

Uses Ristocetin and it unfolds the VWF molecule and reduces repelling negative charges, enabling HMW-VWF multimers to bind reagent platelet membrane GP Ib/IX/V receptors

A

VWF:RCo assay

55
Q

Standard VWD test panel:

May parallel VWF:Ag in subtypes 2A, 2B, and 2M, but are markedly reduced in subtype 2N.

A

Factor VIII assay

56
Q

Von Willebrand Disease Treatment:

A
  • PRICE (protection, rest, ice, compression, elevation)
57
Q

are congenital single-factor deficiencies marked by anatomic soft tissue bleeding

A

Hemophilias

58
Q

used to detect female carriers of hemophilia A

A

Ratio of FVIII activity to VWF:Ag concentration

59
Q

Also called Christmas disease

A

Hemophilia B

60
Q

Also called Rosenthal syndrome, Factor XI deficiency

A

Hemophilia C

61
Q

More than half of the cases have been described in Ashkenazi Jews

A

Hemophilia C

62
Q

patients form weak (non-crosslinked) clots that dissolve within 2 hours when suspended in 5M Urea solution (traditional factor XIII assay)

A

Factor XIII Deficiency

63
Q

Give the INHERITED COAGULOPATHIES and ACQUIRED COAGULOPATHIES of this clotting factor:

Factor I

A

INHERITED COAGULOPATHIES:
- Afibrinogenemia
- Dysfibrinogenemia

ACQUIRED COAGULOPATHIES:
- Severe liver disease
- DIC
- Fibrinolysis

64
Q

Give the INHERITED COAGULOPATHIES and ACQUIRED COAGULOPATHIES of this clotting factor:

Factor II

A

INHERITED COAGULOPATHIES:
- Prothrombin deficiency

ACQUIRED COAGULOPATHIES:
- Liver disease
- Vitamin K deficiency
- Anticoagulant therapy

65
Q

Give the INHERITED COAGULOPATHIES and ACQUIRED COAGULOPATHIES of this clotting factor:

Factor V

A

INHERITED COAGULOPATHIES:
- Factor V deficiency (Owren’s disease/Parahemophilia)

ACQUIRED COAGULOPATHIES:
- Severe liver disease
- DIC
- Fibrinolysis

66
Q

Give the INHERITED COAGULOPATHIES and ACQUIRED COAGULOPATHIES of this clotting factor:

Factor VII

A

INHERITED COAGULOPATHIES:
- Factor VII deficiency

ACQUIRED COAGULOPATHIES:
- Liver disease
- Vitamin K deficiency
- Anticoagulant therapy

67
Q

Give the INHERITED COAGULOPATHIES and ACQUIRED COAGULOPATHIES of this clotting factor:

Factor VIII

A

INHERITED COAGULOPATHIES:
- Hemophilia A
- vWD

ACQUIRED COAGULOPATHIES:
- DIC
- Fibrinolysis

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