Type of von willebrand disease

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Created by
Sarah Jane Glindro

Cards (33)

  • Type of von willebrand disease that is quantitative (2):
    type 1 and type 3
  • type of von willebrand disease that is qualitative:
    Type 2
  • type VWD that is most common approx. 70-80% .
    Type 1
  • lab eval where in:
    Ristocetin cofactor: ↓
    vwf Ag: ↓
    RCO:VWF : N
    FVIII activity: ↓ or N
    RIPA: ↓ or N
    platelet count: N
    Multimer pattern: normal distribution

    Type 1 VWD
  • this are the ff results of lab eval:
    Ristocetin: ↓↓
    VWF ag: ↓↓
    RCO:VWF: N
    FVIII activity: ↓↓
    RIPA: ↓
    platelet count: N
    multimer pattern: absentmultimers

    Type 3
  • The second most common von willebrand disease:
    Type 2A
  • this are the ff lab evaluation:
    Ristocetin: ↓
    vwf ag: ↓
    Rco: VWF: ↓
    FVIII activity: ↓ or normal
    RIPA activity: ↓ or normal
    platelet count: N
    multimer pattern: ↓HMWK
    type 2A
  • this are the ff lab evaluation:
    Ristocetin: ↓
    vwf ag: ↓
    Rco: VWF: N or ↓
    FVIII activity: ↓ or normal
    RIPA activity: ↑ with low dose
    platelet count: ↓
    multimer pattern: ↓HMWK

    type 2B
  • also known as pseudo von willebrand diseasewhich has an increase affinity of platelets to VWF.
    platelet type
  • this are the ff lab evaluation:
    Ristocetin: ↓
    vwf ag: ↓
    Rco: VWF: N
    FVIII activity: N
    RIPA activity: ↑ with low dose
    platelet count: ↓
    multimer pattern: ↓HMWK



    Platelet type
  • mimicking hemophilia A
    Type 2N
  • this are the ff lab evaluation:
    Ristocetin: ↓ or N
    vwf ag: ↓ or N
    Rco: VWF: N
    FVIII activity: ↓
    RIPA activity: N
    platelet count: N
    multimer pattern: N
    type 2N
  • this are the ff lab evaluation:
    Ristocetin: ↓
    vwf ag: ↓
    Rco: VWF: ↓
    FVIII activity: ↓ or normal
    RIPA activity: ↓ or normal
    platelet count: N
    multimer pattern: N
    type 2M
  • produced by platelets and endothelial cells, stored in weibel-palade bodies.
    vWF
  • Platelet aggregation studies undergo shape changes (3):
    increased surface area
    increased receptor expression
    organelle reorganization
  • reversible if stimulus is not sufficient:
    Primary aggregation
  • Irreversible: occurs after internal ADP release, TxA2 synthesis and release

    secondary aggregation
  • sample used in Platelet aggregation studies:
    Platelet Rich Plasma
  • platelet aggregation studies sample prep (4)
    must be tested w/in 4 hrs
    no aspirin intake for 7-10 days before the test
  • N= ECA, Abn= Ristocetin
    disease assoc. (2):

    von willebrand disease
    bernard soulier syndrome
  • giant platelet syndrome
    bernard soulier syndrome
  • ↓ GP IB/ IX/V complex
    bernard soulier syndrome
  • Abn= ECA , N= Ristocetin

    afibrinogenemia
    glanzmann's thrombasthenia
  • ↓GP IIb/IIIa
    glanzmann's thrombasthenia
  • decrease expression of CD41 & CD61
    glanzmann's thrombasthenia
  • partial = ADP & Epinephrine
    abn= Collagen
    N= Ristocetin

    Storage Pool Disorders:
    Chediak Higashi
    Hermansky-pudlak
    Wiskott-Aldrich
  • Defective TXA2 synthesis
    Storage pool disorders
  • characterized by albinism, recurrent infxns, lysosomal granules, dense granules
    Chediak-Higashi
  • dense granules deficiency
    Hermansky-pudlak
  • hermansky pudlak triad of infections:
    tyrosinase positive
    oculocutaneous albinism, accumulation of ceroid- like pigment in macrophages
    bleeding tendency
  • both alpha and dense granules
    Wiskott-Aldrich syndrome
  • wiskott aldrich syndrome triads of infections:
    •thrombocytopenia
    •recurrent infections
    •eczema
  • Dense granules:
    Ca2+
    ADP
    Phosphate
    ATP
    Serotonin