BACKGROUND: The nomenclature for Platelet-type von Willebrand's 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 VWF assays while the disorder is due to pathogenic gain-of-function variants in the platelet GP1BA.

OBJECTIVES: This joint ISTH subcommittees' (SSCs) project aimed to engage with the international community on the disease's nomenclature.

METHODS: A working group, established under the auspice of 3 SSCs, 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 four names GPIBA GoF disorder, PT-VWD, GPIbα GoF disorder and PT-pseudo VWD, were selected in the first survey round by 40-60% of participants. The first two were the top choices and showed almost equal support (28% and 24% respectively). A second focused round did not generate consensus for name change.

CONCLUSIONS: 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.