Evans Syndrome: What to Do When First-Line Corticosteroids Have Not Worked

Clinical scenario

This protocol addresses patients with Evans syndrome who have completed a first-line corticosteroid-based regimen and have failed to reach the established haematological response thresholds for either the immune thrombocytopenic or the autoimmune haemolytic component — or both.

Previous line — failure condition

First-line therapy with corticosteroids (predniso(lo)ne or dexamethasone), with or without IVIg for severe bleeding manifestations or RBC transfusion support for severe wAIHA, did not achieve the required response targets:

Partial response: platelet count ≥ 30 × 10⁹/L with at least a doubling of the initial count, and/or Hb ≥ 10 g/dL with at least a 2 g increase from baseline
Complete response: Hb > 12 g/dL without ongoing haemolysis and platelet count ≥ 100 × 10⁹/L

Next-line approach (partial overview)

The evidence-based step after corticosteroid failure centres on a B-cell depleting monoclonal antibody. In the specific situation where only the thrombocytopenic component remains active and symptomatic, a receptor-targeted agent that supports platelet production is a recognised alternative. The complete regimen — sequencing, dosing, decision criteria — is available in the full protocol.

Instant Access to Structured Evidence-Based Regimens

References

Although rituximab is not licensed for ITP and/or AIHA, its efficacy and good safety profile have been shown throughout randomized prospective studies.

When only itp remains active and symptomatic after corticosteroids rituximab, the use of a thrombopoietin receptor agonist (Tpo-RA) such as eltrombopag or romiplostim is relevant and effective.

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