May 8, 1980New England Journal of Medicine
Heightened Interaction between Platelets and Factor VIII/von Willebrand Factor in a New Subtype of von Willebrand's Disease
Population
20 persons from five families with a qualitative abnormality of Factor VIII/von Willebrand factor showing…
Design
Case_series
Authors
Discussion
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Member takes
Implication
May prompt consideration of Type IIB in atypical von Willebrand presentations; leaves open need for validation in larger cohorts.
Key Points
- To characterize a distinct subtype of von Willebrand's disease presenting with a qualitative abnormality and heightened platelet reactivity.
- Evaluated 20 individuals across five families exhibiting qualitative Factor VIII/von Willebrand factor (FVIII/vWF) abnormalities.
- Assessed platelet-protein interactions using ristocetin-induced platelet agglutination, platelet binding assays, and crossed immunoelectrophoresis of plasma FVIII/vWF.
- Identified a novel variant designated Type IIB, characterized by enhanced ristocetin-induced platelet agglutination and binding, prompting reclassification of the reduced-interaction variant as Type IIA.
- Observed an identical loss of larger, less anodic plasma FVIII/vWF multimeric forms in both Type IIA and Type IIB via crossed immunoelectrophoresis.
Structured PICO
P
Population
20 persons from five families with a qualitative abnormality of Factor VIII/von Willebrand factor (FVIII/vWF) showing heightened responsiveness to ristocetin
O
Outcome
Ristocetin-induced platelet agglutination and binding of FVIII/vWF to plateletssurrogate
Identifies Type IIB von Willebrand's disease, characterized by heightened interaction between platelets and FVIII/vWF in the presence of ristocetin.
Cite This Study
Ruggeri et al. (1980) studied this question.