Pustular mycosis fungoides has a poor outcome: a multicentric clinicopathological and molecular case series
Bibliographic record
Abstract
BACKGROUND: Mycosis fungoides (MF) usually has an indolent course. However, some patients develop more aggressive disease and few prognostic parameters have been identified. Isolated cases of pustular MF (pMF) suggest an unfavourable prognosis. OBJECTIVES: To describe the clinicopathological characteristics and prognostic value of pMF. METHODS: We retrospectively collected data from all patients with MF with histological pustules diagnosed from 2009 to 2020. The outcomes and clinicopathological characteristics of pMF at diagnosis (pMFD) were compared with those of a cohort of patients with nonpustular MF (NpMF). RESULTS: Thirty-three patients with pMF (including 22 with pMFD) and 86 with NpMF were included. Median age at diagnosis of pMF was 61 years [interquartile range (IQR) 50-75]. The median duration of follow-up for patients with pMFD was 32 months (IQR 14-49). Clinically, 33% of patients with pMF had pustules. Large cell transformation (LCT) occurred in 17 patients. Patients with pMFD had significantly more advanced-stage disease and showed more LCT at diagnosis than those with NpMF [50% vs. 7% (P < 0.001) and 23% vs. 0% (P < 0.001), respectively]. On multivariate Cox analysis, the presence of histological pustules at diagnosis was associated with shorter overall survival (OS) in all patients [hazard ratio (HR) 13.90, 95% confidence interval (CI) 2.40-79.00); P = 0.003] and in patients with early-stage disease (HR 11.09, 95% CI 1.56-78.82; P = 0.02). In multivariate Fine and Gray model analysis, pMFD was associated with a higher cumulative incidence of LCT (subdistribution HR 13.90, 95% CI 2.43-79.00; P = 0.003) in all patients. Median OS after the occurrence of histological pustules during follow-up in all patients with pMF was 37 months, with a 5-year OS rate of 25% (95% CI 0.06-0.50). CONCLUSIONS: pMF often follows an aggressive course, with a high risk of LCT and shorter survival, even for patients with early-stage disease. Histological pustules at diagnosis of MF might represent an independent poor prognostic factor, to be confirmed by further studies. As pustules are not always identified clinically, pustules found on histology should be mentioned in MF pathology reports and should prompt discussion of closer follow-up.
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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 | 0.002 |
| Meta-epidemiology (narrow) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.002 | 0.001 |
| Science and technology studies | 0.002 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.002 | 0.001 |
| Insufficient payload (model declined to judge) | 0.001 | 0.000 |
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".