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S3B5 Pathology > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

What is the most common inherited bleeding disorder?

A

von Willebrand Disease

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2
Q

Petechiae, purpura or ecchymoses suggest what type of bleeding disorder?

A

thrombocytopenia

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3
Q

Enlarged spleen, liver or lymph nodes suggest what type of bleeding disorder?

A

chronic infections or malignancies

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4
Q

Telangiectasias suggest what type of bleeding disorder?

A

Osler-Weber-Rendu

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5
Q

Hyperextensible joints or paper-thin scars suggest what type of bleeding disorder?

A

Ehlers-Danlos

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6
Q

Skin plaques or scalloped tongue suggest what type of bleeding disorder?

A

amyloid

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7
Q

Musculoskeletal abnormalities suggest what type of bleeding disorder?

A

hemophilia

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8
Q

What is the mutation seen in vWD?

A

quantitative or qualitative deficiency of von Willebrand factor

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9
Q

What clinical symptoms is characteristic of vWD?

A

mucocutaneous bleeding

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10
Q

What is the function of vWF?

A

carrier protein for factor VIII
ligand to bind GPIB receptor on platelets for initiation of platelet adhesion to damaged blood vessel

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11
Q

Where is vWF made?

A

endothelial cells & megakaryocytes

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12
Q

Where is Factor VIII produced?

A

liver & kidney

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13
Q

What are the 3 main types of vWD?
Which type is most common?

A
  • Type 1 - MC (80%)
    • heterozygous or partial quantitative deficiency vWF
    • mild disease
  • Type 2
    • abnormal vWF produced
    • variable severity
  • Type 3
    • homozygous
    • absent or severe deficiency vWF
    • plasma levels factor VIII severely decreased
    • clinically looks like hemophilia A
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14
Q

How do the different types of vWD in the multimer analysis?

A
  • Type 1: all multimes, but reduced quantity
  • Type 2: variable
  • Type 3: none
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15
Q

What is the cause of Hemophilia A?

What are the different severities?

A

deficiency of factor VIII

severe <1%

moderate 1-5%

mild >5%

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16
Q

Hemophilia A has what type of inheritance pattern?

A

X-linked recessive

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17
Q

What are the clinical symptoms of a patient with Hemophilia A?

A

bleeding into joints

hemophilic arthropathy

bleeds with minor trauma

NO mucocutaneous bleeding

18
Q

What lab values would you expect to see in a patient with Hemophilia A?

A
  • prolonged PTT
    • complete correction with mixing study
  • genetic testing - prenatal testing
  • normal PT, TT & bleeding time
    • extrinsic & common pathway are in tact
    • platelet function & vascular integrity are also in tact
19
Q

What is the treatment for Hemophilia A?

A

DDAVP mild cases

factor VIII concentration moderate to severe disease

20
Q

What are possible complications to treatment of Hemophilia A?

A

inhibitors factor VIII

HIV, Hepatitis, Parvovirus B19 (b/c acquired form transfusion)

21
Q

What is the mutation in Hemophilia B?

It has what type of inheritance pattern?

A

deficiency factor IX

X-linked recessive

22
Q

What is the most common cause of acquired factor deficiency?

A

Liver disease

23
Q

Vitamin K is required for function of what factors?

A

factors

II, VII, IX, X

protein C & protein S

24
Q

Normal hemostasis has what 3 components?

A
  1. vascular wall integrity
  2. Platelets (adequate # & function)
  3. coagulation cascade
25
Q

What is Virchow’s Triad?

A

alone or in combo predisposes to thrombosis

  • Endothelial injury
  • Hypercoaguability
  • Abnormal blood flow
26
Q

What situations would warrant a complete thrombophilia workup?

A

thromboembolic event under age 50

unprovoked event at any age

27
Q

What mutation causes Factor V Leiden?

A

point mutation factor V that makes it resistant to proteolytic action of activated protein C

protein C normally degrades activated factor V & factor VIII

28
Q

What is the most common hereditary predisposition to venous thrombosis?

A

Factor V Leiden

29
Q

Individuals homozygous for Factor V Leiden have what relative risk of developing venous thrombosis?

A

80x

30
Q

Prothrombin gene mutation is associated with what molecular abnormality?

A

elevated levels of prothrombin & subsequent increased risk of thrombosis

increased levels TAFI- protein that inhibits clot lysis, so clots last longer

31
Q

Factor VIII levels what percent of normal are associated with increased risk of venous & arterial thrombosis?

This is often seen in what situation?

A

150%

inflammatory states (acute phase reactant)

32
Q

What are the 3 common risk factors for venous thromboembolism?

A

factor V Leiden

prothrombin gene mutation

elevated factor VIII

33
Q

What test do you perform if you suspect Factor V Leiden?

A

screen: activated protein C resistance

genetic testing is definitive

34
Q

Deficiency in Protein C increases risk of DVT in what specific locations?

A

legs & mesenteric veins

pulmonary embolism

35
Q

Protein C & Protein S deficiency are associated with what warfarin-related issue?

A

warfarin-induced skin necrosis

36
Q

What is the clinical presentation of neonates with severely decreased levels of protein C?

A

DIC

purpura fulminans neonatorum

37
Q

What are acquired increases in protein C that may mask deficiencies?

A

ischemic heart disease

pregnancy

post-menopausal state

hormone replacement therapy

oral contraceptives

38
Q

Patients with deficiencies in protein C, protein S & antithrombin often have a thromboembolic even before what age?

A

45

39
Q

How long after a thrombotic event should you wait before thrombophilia testing?

A

2 weeks

40
Q

What thrombophilia tests are not affected by anticoagulants or active thrombosis?

A
  • activated protein C resistance (APCR)
  • homocysteine
  • anticardiolipin antibodies
  • genetic tests for V leiden and prothrombin gene
41
Q

What factors predispose to venous thrombosis?

A
  • factor V Leiden
  • prothrombin mutation
  • elevated VIII levels
  • increased levels II, IX, XI
  • deficiencies protein C, S
42
Q

What factors predispose to arterial thrombosis?

A
  • heparin platelet antibodies
  • hyperhomocysteinemia
  • antiphospholipid antibodies
  • elevated levels factor I, V, VII, vWF
  • deficiency antithrombin

S3B5 Pathology (13 decks)

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