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Updated: Dec 18 2021

Glanzmann Thrombasthenia

  • Snapshot
    • A 2-month-old female baby is seen in the emergency room with frequent nosebleeds and bruising. Her birth history is unremarkable, except for delayed separation of the umbilical cord. On physical exam, there is some blood in his nose. Lab results show normal PT and PTT but increased bleeding time. Her platelets come back at a normal range. Peripheral blood smear shows no clumping of platelets.
  • Introduction
    • Impaired platelet aggregation from genetic deficiency of GpIIb/IIIa
    • Key lab finding
      • normal platelet count
      • ↑ bleeding time
    • Pathogenesis
      • ↓ GpIIb/IIIa (a platelet integrin)
        • recall that GpIIb/IIIa is a receptor on platelets
        • fibrinogen interacts with GpIIb/IIIa between two platelets, helping platelet aggregation
        • recall abciximab inhibits this receptor
      • defect in platelet plug formation
    • Genetics
      • autosomal recessive
    • Associated conditions
      • leukocyte adhesion deficiency commonly seen
        • impaired wound healing
        • delayed umbilical cord separation > 30 days
        • recurrent skin and mucosal infections
      • acquired Glanzmann thrombasthenia
        • in those who received multiple platelet transfusions
  • Presentation
    • Symptoms from platelet abnormalities
      • mucocutaneous microhemorrhages
        • bleeding from mucous membranes
        • epistaxis
        • petechiae/purpura
    • ↑ bleeding time
    • Normal PT/PTT
    • Complete blood count
      • normal platelet count
      • normal platelet morphology
    • Peripheral blood smear
      • no platelet clumping seen
  • Differential Diagnosis
    • Bernard-Soulier (↓ platelet count, ↑ bleeding time, and ↓ GpIb)
    • ITP (↓ platelet count, ↑ bleeding time, and anti-GpIIb/IIIa antibodies)
    • TTP (↓ platelet count, ↑ bleeding time, and defective/deficient ADAMTS13)
  • Treatment
    • Prevention
      • Avoid anti-platlet agents (aspirin and NSAIDs)
    • Platelet transfusions
      • with HLA-matched and leukocyte depleted blood products
    • No clear evidence but options are
      • recombinant factor VIIa
      • hematopoietic stem cell transplant
    • Supportive care
      • iron and folate supplementation
  • Complications
    • Uncontrolled bleeding
  • Prognosis
    • Good with supportive care
    • Generally, bleeding decreases with age
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