International opinion survey on nomenclature for platelet-type von Willebrand disease: communication from the SSC of the ISTH
Bibliographic record
Abstract
BACKGROUND: The nomenclature for platelet-type von Willebrand disease (PT-VWD) remains an open debate, with several proposed names in use in the literature for this rare inherited platelet disorder. It has been suggested that this name may lead to delays in diagnosis since the diagnostic pathway of patients often starts with von Willebrand factor assays, while the disorder is due to pathogenic gain-of-function (GOF) variants in the platelet GP1BA. OBJECTIVES: This joint International Society on Thrombosis and Haemostasis (ISTH) Subcommittees' project aimed to engage with the international community on the disease's nomenclature. METHODS: A working group, established under the auspices of 3 Subcommittees, explored issues around nomenclature and proposed possible names with input from a panel of 6 global experts. Two rounds of surveys were administered online via ISTH REDCap and My ISTH Community. RESULTS: The 4 names, GPIBA GOF disorder, PT-VWD, glycoprotein Ibα GOF disorder, and PT-pseudo VWD, were selected in the first survey round by 40% to 60% of participants. The first 2 were the top choices and showed almost equal support (28% and 24%, respectively). A second focused round did not generate consensus for name change. CONCLUSION: Based on the survey results, the decision was in support of retention of the more commonly used nomenclature (PT-VWD). More work is needed to ensure this name is adopted universally and in all current online databases to achieve consistency.
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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 | 0.015 | 0.042 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.002 | 0.001 |
| Bibliometrics | 0.005 | 0.006 |
| Science and technology studies | 0.002 | 0.003 |
| Scholarly communication | 0.004 | 0.005 |
| Open science | 0.003 | 0.003 |
| Research integrity | 0.008 | 0.010 |
| Insufficient payload (model declined to judge) | 0.007 | 0.004 |
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".