Module overview
Section 5 of 6 · Open sections

Required section · Section 5 of 6

Learner decision

Use the whole pattern to localize four failure points. Immune thrombocytopenia, abbreviated ITP, is quantitative platelet loss without a primary defect in adhesion or aggregation of surviving platelets. VWD is a VWF-mediated adhesion problem that requires laboratory characterization. Bernard-Soulier syndrome affects GPIb-IX-V and typically combines thrombocytopenia, giant platelets, and reduced ristocetin-induced agglutination.

Glanzmann thrombasthenia affects αIIbβ3, so aggregation to ADP, epinephrine, and collagen is absent while ristocetin-induced agglutination is normal. Thrombin also aggregates through αIIbβ3 but is not part of the routine LTA agonist panel used here. In the guided case, normal count and morphology make Bernard-Soulier syndrome less consistent, while reduced ristocetin makes Glanzmann thrombasthenia less consistent. The preferred action is not to report a subtype from LTA alone. Order the VWF studies that can characterize the VWF-mediated finding.

Sequence also matters. A platelet cannot aggregate through fibrinogen bridging before activation increases αIIbβ3 affinity, and activation follows adhesion to the exposed matrix. Keep the sequence visible when comparing defects. Choose the next test that separates the remaining differential rather than repeating evidence that already agrees.

Illustrative drawing — this picture was drawn rather than captured.

Four defect cards list key count, morphology, and ristocetin patterns for ITP, VWD, Bernard-Soulier syndrome, and Glanzmann thrombasthenia.
Figure 1Four cards compare quantitative platelet loss with VWF-mediated adhesion, GPIb-IX-V, and αIIbβ3 defects.

Ordering exercise

Place the primary-hemostasis events in the order that supports a temporary platelet plug.

  1. 1. Fibrinogen bridges activated αIIbβ3

    Adjacent activated platelets aggregate.

  2. 2. ADP and thromboxane A2 recruit platelets

    Activated platelets change shape and recruit additional platelets.

  3. 3. Vessel injury exposes matrix

    Loss of the intact endothelial barrier exposes collagen and VWF.

  4. 4. VWF tethers platelets through GPIb-IX-V

    The initial high-shear interaction brings platelets to the injured site.

Knowledge checks

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Knowledge check 1

Which defect most classically preserves ristocetin-induced agglutination while other agonist responses are absent?

Choose one option.

Knowledge check 2

Select the observations that make Bernard-Soulier syndrome less consistent with the guided case.

Choose at least 2 options.

Section status

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