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Coagulation > Clinical Conditions Associated with Abnormal Hemostasis > Flashcards

Clinical Conditions Associated with Abnormal Hemostasis Flashcards

1
Q

how common is Von Willebrands Disease?

A

most common inherited bleeding disorder in North America

1-2% of general population

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2
Q

how do you get Von Willebrand’s Disease?

A

autosomal trait

recessive trait

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3
Q

what does Von Willebrand’s Factor do?

A
  • mediate platelet adhesion at sites of vascular injury

- binds to and protects Factor VIII from in-vivo proteolysis (the breakdown of protein into amino acids)

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4
Q

deficiency of VWFactor affects

A

primary hemostasis

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5
Q

what percentage is a mild form of deficiency of VWFactor

A

> 40% VWF

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6
Q

deficiency of VWFactor is associated with

A

mucosal bleeding

  • epistaxis
  • menorrhagia
  • bruising
  • oral cavity
  • GI bleeding
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7
Q

more severe forms of of deficiency of VWFactor

A
  • decrease VWF and decrease Factor VIII
  • severe mucosal bleeding
  • bleeding into joints and muscles
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8
Q

forms of severe deficiency of Factor VIII

A
  • spontaneous joint bleeds
  • retoperitoneal hemorrhage (behind peritoneal: spleen, spine, part of aorta)
  • resembles hemophilia
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9
Q

treatment of for deficiency of VWF/Factor VIII

A

DDAVP (in mild cases): Desmopressin, Stimulating release of the VWF from its storage sites in endothelial cells. Cleaving the large VWF multimers circulating in plasma into smaller multimers.

  • plasma products that contain VWF
  • adjunct treatments such as anti-fibrinolytic (Amicar) and estrogen
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10
Q

what are some plasma products that contain VWF

A
  • cryoppt
  • FVIII/VWF concentrates
  • purified VWF
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11
Q

what are congenital plasma coagulation deficiencies

A
  • hemophilia A & B (Factor VIII and IX deficiency)

- Factors XI, X, V, II and I (Fibrinogen)

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12
Q

how is Hemophilia A & B acquired?

A
  • X-linked recessive disorders (woman carries it)
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13
Q

Severity of bleeding for hemophilia A and B

A

factory activity level

  • <1% activity - spontaneous bleeds
  • 1-5 % - post-operative and bleeding with minor trauma
  • 6-20% - post-operative and with trauma
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14
Q

treatment hemophilia A and B

A
  • replacement with specific factor concentrates

- avoid platelet inhibitory drugs

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15
Q

treatment for Factor XI, X, V, II and I (Fibrinogen) deficiencies

A
  • replacement transfusion with FFP

- no platelet inhibitory drugs

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16
Q

Congenital Plasma Coagulation Deficiencies NOT associated with abnormal bleeding

A
  • Factor XII deficiency (ex: Hageman - intrinsic not really activated)
  • HMW Kininogen Deficiency (Fitzgerald Factor)
  • Prekallikrein Deficiency (Fletcher Factor)
17
Q

Acquired Disorders of Plasma Coagulation

A
  • liver disease
  • renal disease (nephrotic syndrome)
  • vitamin K deficiency
  • DIC (disseminated intravascular coagulation)
  • Antiphospholipid Antibodies (Lupus Anticoagulant)
  • Acquired antibodies to coagulation factors
18
Q

how does Coumadin (Warfarin) work?

A

binds to albumin

blocks factor 2, 7, 9, 10

19
Q

what are some congenital intrinsic Platelet function abnormalities

A
  • Glanzman’s Thrombasthenia
  • Bernard Soulier
  • Storage Pool Diseases
20
Q

what is the most common of the congenital intrinsic platelet function abnormalities?

A

Bernard Soulier

21
Q

what is another name for Storage Pool Disease?

A

Gray Platelet Syndrome

comes from platelets not kept moving in storage

22
Q

thrombophilia

A

hereditary or acquired disorders that predispose an individual to thrombosis

23
Q

Congenital Abnormalities under Thrombophilia

A
  • Factor V Leiden Mutation
  • Protombin 20210G Mutation
  • AT3 Deficiency
  • protein C/S Deficiency
24
Q

what is the most common congenital abnormality and most common acquired abnormality

A

AT3 deficiency

25
Q

Out of Protein C/S deficiency, what is the most important?

A

Protein C

- S supports C

26
Q

what does Protein S regulate? what does it inactivate and what does it NEED?

A

regulate so you don’t over clot

  • inactivate F5, 8, 10, 2
  • NEEDS vitamin K
27
Q

Risk factors for Thrombosis

A
  • myeloproliferative disorders
  • metabolic disorders such as hyperhomocysteinemia
  • malignancy
  • diabetes
  • antiphospholipid antibodies
  • microangiopathic disorders such as DIC and TTP/HUS
  • Type 2 heparin induced thrombocytopenia (HIT)
28
Q

Other risk factors that thrombosis can affect

A
  • hormones
  • oral conctraceptive
  • pregnancy
  • vasculitis
  • smoking
  • stasis (stagnet blood)
  • obesity
29
Q

Lab assessment of thrombosis

A
  • antiphospholipid antibodies
  • Quantitation of Inhibitors: AT3, Protein C/S, Tissue Factor Pathway
  • Activated Protein C Resistance: Factor V Mutation
  • Factor II Mutation

Coagulation (5 decks)

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