April 1, 1977British Journal of Haematology
Thromboxane Synthesis and the Platelet Release Reaction in Bernard‐Soulier Syndrome, Thrombasthenia Glanzmann and Hermansky‐Pudlak Syndrome
Population
5 cases with characterized platelet disorders
Comparison
In vitro platelet stimulation with collagen… vs Normal platelets
Design
Case_series
Authors
Discussion
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Implication
Clinicians should consider non-TX pathways when evaluating these syndromes; case report leaves open primary defects for mechanistic research.
Structured PICO
P
Population
5 cases with characterized platelet disorders (1 Bernard-Soulier syndrome, 1 Hermansky-Pudlak syndrome, 3 Glanzmann's thrombasthenia)
I
Intervention
In vitro platelet stimulation with collagen, ADP, arachidonic acid, or prostaglandin G2 (PGG2)
C
Comparator
Normal platelets
O
Outcome
Thromboxane (TX) synthesis, release of ADP or [14C]serotonin, and platelet aggregationsurrogate
The study demonstrates that platelet dysfunction in Bernard-Soulier syndrome, Hermansky-Pudlak syndrome, and Glanzmann's thrombasthenia is not caused by defective thromboxane synthesis from arachidonic acid.
Cite This Study
Malmsten et al. (1977) studied this question.