Blastic plasmacytoid dendritic cell neoplasm (BPDCN): international, multi-center collaboration and global registry program
Bibliographic record
Abstract
BACKGROUND: Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a rare and aggressive hematologic malignancy originating from plasmacytoid dendritic cell precursors. BPDCN shares common diagnostic and clinical features with other hematologic malignancies and various dermatological disorders. Differential diagnosis and treatment are challenging and require awareness by the dermatologist, hemato-oncologist, pathologist for detailed diagnostic and therapeutic workup. The outcomes remain poor, and the optimal treatment for the disease is yet to be established, emphasizing the need for a more comprehensive and globally inclusive approach. METHODS: In response to these challenges, we initiated an international, multi-center collaboration and established a global registry program for BPDCN patients. The registry collected both retrospective and prospective data on demographics, clinical presentations, diagnostic criteria, treatment regimens, and outcomes for cases diagnosed after Jan 2010. Data for this report was obtained from 36 patients across 16 centers in 12 countries, with ongoing contributions from additional centers. RESULTS: Preliminary analysis revealed a male predominance (78%), with a median age at diagnosis of 63 years, involving all age groups. The immunophenotype (CD123 + , CD4 + , CD56 +) was consistently observed in a majority of patients (88.8%), validating its diagnostic utility and paramount significance in the BPDCN diagnosis. Treatment responses varied based on initial regimens, with ALL-like approaches demonstrating more favorable outcomes compared to AML-like strategies, which were given to younger patients. Notably, relapse rates remained high. CONCLUSION: The BPDCN International Registry Program provides a valuable tool in consolidating global data and fostering collaboration among researchers and clinicians. This collaborative effort involving multiple countries on several continents not only aims to advance our comprehension of BPDCN but also lays the groundwork for standardized treatment protocols for improving outcomes for BPDCN patients worldwide.
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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 | 0.012 | 0.012 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.004 | 0.007 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.002 |
| Open science | 0.001 | 0.004 |
| Research integrity | 0.000 | 0.001 |
| Insufficient payload (model declined to judge) | 0.003 | 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".