Relapse pattern and long‐term outcomes in subjects with acquired haemophilia A
Bibliographic record
Abstract
INTRODUCTION: Acquired haemophilia A (AHA) is a rare autoimmune bleeding disorder caused by neutralizing antibodies against factor VIII (FVIII). Despite significant initial morbidity and mortality, most patients achieve remission with immunosuppressive therapy. AIM: Long-term follow-up data from the Quebec Reference Centre for Inhibitors (QRCI) were analysed to identify factors predictive of AHA relapse and the influence of relapse on survival. METHODS: Criteria used to define AHA were levels of FVIII <0.3 IU/mL and FVIII inhibitor titres ≥0.6 Bethesda Units (BU). Complete remission was defined as FVIII >0.5 IU/mL and/or FVIII inhibitor titres <0.6 BU while not on immunosuppression. RESULTS: Between 2000 and 2012, 111 subjects met the inclusion criteria and were followed for a median of 25.6 months. Ninety per cent of them reached remission on immunosuppression in a median time of 45 days. Fourteen patients presented one or more relapses in a median time of 13.4 months. Most relapse episodes were successfully treated. Associated lymphoproliferative syndromes (LPS) were predictive of relapse, whereas FVIII activity and inhibitor titres at initial diagnosis or immunosuppressive regimens were not. The overall survival (OS) was the same, with or without relapse. CONCLUSION: Among the recognized potential risk factors for relapse, only LPS was statistically significant. The long-term follow-up of our patients also showed that late or multiple relapses may occur, but that relapse is not associated with a worse OS. Thus, long-term follow-up is important for optimal management of AHA.
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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 | 0.000 | 0.001 |
| 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 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 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".