Cold agglutinin disease (CAD) is a haemolytic anaemia requiring a staged treatment strategy. When initial rituximab-based therapy does not achieve its intended haematological and clinical goals, a defined next-line protocol is indicated.
First-line treatment with rituximab — either as monotherapy or in combination with bendamustine — targets an increase in haemoglobin levels, transfusion independence, and improvement or resolution of cold-induced circulatory symptoms. Response in CAD may take one to eight months. When these goals are not reached, escalation to the next protocol is warranted.
This protocol uses complement inhibition administered by intravenous infusion. The specific agent, selection criteria, and administration schedule are available in the full protocol.
The protocol targets inhibition of haemolysis within 24 hours, increased mean haemoglobin levels within the first week, achievement of transfusion independence, and normalisation of bilirubin by week 1.