Treatment of Multi-Refractory Evans Syndrome
When Evans syndrome does not respond adequately across multiple lines of therapy, a distinct clinical approach is required. This protocol addresses the multi-refractory setting — where prior treatment has failed to achieve or sustain adequate disease control.
This protocol applies specifically to patients with multi-refractory Evans syndrome, a challenging course in which the condition has not been controlled by earlier therapeutic attempts.
Partial overview: For multi-refractory Evans syndrome, one approach involves agents such as bortezomib combined with dexamethasone — though additional options exist. The full evidence-based regimen, selection criteria, and complete treatment algorithm are available only in the structured protocol below.
References
For patients with multi-refractory ES, targeting long-lived autoreactive plasma cells by means of bortezomib combined with dexamethasone or daratumumab may be a good option.
Another option in the future could also be to use fostamatinib, a spleen tyrosine kinase (syk) inhibitor that has been approved for adult' primary ITP and that has also shown a promising efficacy for wAIHA in a phase II trial.
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