Results of a World Wide Survey On the Diagnostic Dilemma Between Type 2B Von Willeband Disease and the PT-VWD.
Bibliographic record
Abstract
Abstract Abstract 4436 The Canadian PT-VWD project (www.pt-vwd.org) initiated in 2007 is based on a molecular diagnostic approach to differentiate between type 2B VWD and its closely similar disorder PT-VWD. Recruitment of worldwide samples and data still poses a challenge partly because of the relative rarity of the two bleeding disorders and more importantly because it is dependant on the level of awareness about the requirements for the differential diagnosis and the subsequent impact on the treatment decision. The objective of this survey was to gather an opinion from the worldwide hemostasis community about this diagnostic dilemma and the current utilization of the appropriate diagnostic tests. The survey comprised eight key questions (see link on website) sent individually by email to hematologists from different countries and representing a variety of academic centres. Out of 60 emails we received 37 responses. The highest response was from Europe 16/37 followed by USA 9/37. Other responses were from Africa (3), Canada (2), Asia (2), Middle East (2), South America (1), Mexico (1) and Australia (1). Fifty four percent of specialists have seen or diagnosed 1-5 type 2B VWD cases and 38% have seen or diagnosed 1-5 PT-VWD cases. 82% have reported they have access to laboratory phenotypic tests including RIPA and/or platelet mixing studies with only 59% reporting they have access to genetic testing of the two responsible genes: VWF and platelet GP1BA gene. Genetic analysis was the most discriminative test in the opinion of 54% and the RIPA was chosen to be the discriminative test by 35% while 11% believed the appropriate discrimination should be based on both. 84% agreed to classify PT-VWD under platelet function disorders and 73% have felt that PT-VWD should be investigated when platelet function defects are suspected. On a scale from 1-10 with 10 being most important, 30/37 of participants reported 7-10 to rate the importance of this diagnostic dilemma and 13% have reported 5. Despite the relatively small number of respondents, the data obtained through this survey confirm the rarity of the two disorders, reflects the positive world-wide opinion re the diagnostic challenges of the two closely similar disorders and indicate the significant unavailability of genetic analysis as a useful confirmatory test. Disclosures: No relevant conflicts of interest to declare.
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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 | 0.003 | 0.009 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.000 | 0.001 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.002 | 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".