PB2647: USE OF EMICIZUMAB FOR THE TREATMENT OF ACQUIRED HEMOPHILIA A: A SYSTEMATIC REVIEW
Bibliographic record
Abstract
Topic: 33. Bleeding disorders (congenital and acquired) Background: Acquired Hemophilia A (AHA) is a rare disease that occurs due to the development of antibodies against FVIII, leading to severe bleeding complications with high morbidity and mortality. Emicizumab is a bispecific, monoclonal antibody that bridges activated factor IX (FIXa) and X (FX). It has shown to be effective in achieving hemostatic control in people with hemophilia A with FVIII inhibitors. Thus, it has been used off-label in the management of AHA. Aims: We conducted a systematic review on the use of Emicizumab for the treatment of AHA, focusing on the time required to achieve bleeding control. Methods: We conducted a comprehensive search in PubMed, EMBASE, Medline and The Cochrane Library from inception until January, 2023. Studies reporting cases of patients diagnosed with AHA that received at least one dose of emicizumab were included (Fig 1). Results: Eighteen studies were included in the final review for a total of 45 patients. The median age was 78 years (range: 21 – 93). The vast majority (75.6%) were males. Presenting bleeding symptoms were widely variable; the most common were subcutaneous bleeding and muscle hematomas. In forty patients the FVIII levels at diagnosis were ≤ 1% or undetectable. The median inhibitor level was 40 BU/mL (1.8 to 2000 BU/mL). Kaplan-Meier analysis showed that around 56% of patients have been reported to achieve bleed control in one day after the start of treatment and almost 90% of patients had no bleeding by day 5. (Fig 2). Side effects were infrequently reported; nevertheless, the treatment appeared to be well-tolerated Summary/Conclusion: Emicizumab appears to be an effective and safe hemostatic treatment for AHA. Bleeding control is rapidly achieved after initiating emicizumab. Clinical trials comparing emicizumab versus standard hemostatic agents are needed to confirm these findings.Keywords: Acquired factor VIII inhibitor, Acquired hemophilia
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.006 | 0.002 |
| Bibliometrics | 0.000 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 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 teacher head, 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".