Treatment of Chronic Lymphocytic Leukemia with Autoimmune Haemolytic Anaemia or Immune Thrombocytopenia

Autoimmune complications — specifically autoimmune haemolytic anaemia (AIHA) and immune thrombocytopenia (ITP) — occur in a clinically meaningful proportion of patients with chronic lymphocytic leukaemia (CLL) and require specific management guidance distinct from the underlying leukaemia itself.

Clinical Scenario

This protocol addresses CLL complicated by autoimmune haemolytic anaemia (AIHA) or immune thrombocytopenia (ITP). These autoimmune cytopenias are relatively frequent in CLL (5%–7%), and patients with CLL-associated autoimmune cytopenias should be managed in the same way as those with primary AIHA or ITP.

Treatment Approach

Treatment may include rituximab monotherapy, with further targeted options available depending on the specific autoimmune complication and whether the presentation is refractory.

Full regimen details, sequencing, and options for refractory cases are available in the complete protocol below.

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References

Autoimmune haemolytic anaemia (AIHA) and, to a lesser extent, immune thrombocytopenia (ITP), are relatively frequent in CLL (5%–7%).

CLL patients with simple AIC should be managed in the same way as patients with primary AIHA or ITP (GRADE 1D).

For patients not responding to corticosteroids, rituximab monotherapy and/or thrombopoietin agonists in the case of ITP (e.g. romiplostim, eltromboplag, avatrombopag) are recommended second-line options.

For refractory cases, complement inhibition with sutimlimab (anti-C1S monoclonal antibody) is an effective option.

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