PB0838 Association between Hemoglobin Values and VWF Assays: A Multi-Center Investigation
Bibliographic record
Abstract
Aims: We addressed the question of whether a previously uncharacterized platelet dysfunction contributes to bleeding diathesis in 2B-VWDMPS patients.Methods: We studied the 2 members (mother and daughter) of the MPS kindred with severe bleeding phenotype and addressed this question by coupling quantitative platelet shotgun proteomics and validating biochemical assays, with the systematic analysis of platelet procoagulant membrane dynamics (PMD).Results: Previously, we showed that membrane ballooning, a principal part of the platelet PMD after collagen stimulation, is driven by the influx of Na+ and Cl-, followed by the entry of water.Here, we report in 2B-VWDMPS platelets, loss of the transmembrane chloride channel-1 (CLIC1), and reduced chloride ion influx after collagen stimulation.This was associated with diminished membrane ballooning, phosphatidylserine externalisation and membrane thrombin formation, as well as a distinct phenotypic composition of platelets over fibrillar collagen.Using N-terminomics/ TAILS (terminal amine isotopic labeling of substrates), we compared changes in proteolysis between healthy and 2B-VWDMPS platelets and identified processing differences of von Willebrand factor (VWF), fibronectin (FN1) and Crk-like protein (CRKL).Conclusion(s): 2B-VWDMPS platelets are basally activated, partially degranulated, and have marked loss of regulatory, cytoskeletal, and contractile proteins that may account for structural disorganization, giant platelet formation and the reduction in the haemostatic response.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.001 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 0.000 |
| Scholarly communication | 0.001 | 0.000 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.004 | 0.001 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".