Disorders of Primary Hemostatic function

Describe the different types of bleeding patients may present with and correlate this to the hemostatic mechanism most likely dysfunctioning

Primary

• Skin and mucous membranes

  • Purpura

  • Petechiae

  • Ecchymoses

  • Epistaxis

  • Menorrhagia

Secondary

• Deeper tissues

  • Hemartjrosis

  • Hematuria

Define and Differentiate petechiae, ecchymosis, and hematoma

Petechaie

• Similar to a rash

Ecchymosis

• A bruise

Hematoma

• Fluid acclimation

Correlate clinical findings and screening tests results to the most likely source of primary hemostatic dysfunction

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Outline the diagnostic work-up required for a patient presenting with bleeding

Background information

Lab test

• CBC

• PT

• aPTT

List the two major problems that occur to the vasculature when a hereditary defect is present in a patient

weakened vessel walls and abnormal vessel development

Associate the hereditary defect of the vasculature with its impact on normal blood vessel integrity

List the four etiologies of acquired disorders of vascular function

Define thrombocytopenia and thrombocytosis

List the three major etiological groups of thrombocytopenia

Impaired production

Increased destruction

Distribution abnormality

Describe the etiology, pathophysiology, clinical features, and laboratory findings of the following quantitative defects of platelets:

Idiopathic thrombocytopenic purpura

thrombotic thrombocytopenic purpura

Post-transfusion purpura

Disseminated intravascular coagulation

Hemolytic uremic syndrome

Neonatal alloimmune thrombocytopenia

Heparin-induced thrombocytopenia

Drug-induced immune thrombocytopenia

Describe the etiology, pathophysiology, clinical features, and laboratory findings of the following quantitative defects of platelets:

von Willebrand’s disease

Bernard-Soulier syndrome

• Large Platelet disease

  • Mutation in gene that codes for GPIb, Chromosome 17 and 22, GP IX, Chromosome 3

  • inappropriate adhesion to collagen

• Manifested in infancy or childhoos

  • severe bleeding (seldom)

  • Brusing

  • epitaxis

  • gingivial bleeding

• Lab Values

  • prolong bleeding time (20 minutes)

  • platelet count decreased (50,000 - 80,000)

  • giant platlets

  • platlet aggregation

    • Absent with thrombonin

    • absent with bovine vWF or ristocetin

    • normal ADP, collagen and epinephrine

Glanzmann’s thrombasthenia

• Autosomal recessive inheritance pattern

  • Mutation in GPIIb, chromosome 17, GP IIIa, chromosome 17

    • platlet aggregation can not occur

  • appear in infancy or childhood

  • severe and debilitating bleeding episodes

• Lab values

  • Normal platelet count

  • prolong bleeding time

  • platelet aggregation

    • no complete response to agonist

Storage pool deficiencies

• Dense granule def.

• Alpha granule def

• Other

Describe and differentiate the different subtypes of vWF

Describe the different laboratory tests used to examine vWF activity

Correlate platelet aggregation results to Bernard-Soulier Syndrome, vonWillebrand Disease, Glanzmann Thrombasthenia, and storage pool disorders

Determine the most likely defect of primary hemostasis when provided a case study