The 2026 AustrAIHANZ consensus from Australia and New Zealand gives clinicians a practical approach to suspected autoimmune haemolytic anaemia (AIHA). The diagnostic work-up considers warm, cold and mixed disease; the treatment pathway discussed here primarily concerns warm AIHA. The key decisions are whether haemolysis is immune, whether anaemia requires urgent support, and when to introduce a steroid-sparing treatment.
Establish haemolysis, then interpret the DAT
Start with the haemoglobin and reticulocyte count, blood film, LDH, unconjugated bilirubin and haptoglobin. Spherocytes and polychromasia can support warm haemolysis, while agglutination suggests a cold antibody. Read the direct antiglobulin test (DAT) in that clinical context: a positive result can occur after transfusion or immunoglobulin-based treatment without establishing AIHA, and a negative result does not rule it out. If suspicion persists, discuss more sensitive or extended testing with the laboratory.
The panel recommends a polyspecific DAT followed by monospecific testing to help classify the disease (1D). IgG with or without C3d often supports warm AIHA. C3d alone should prompt evaluation for cold-mediated disease. Dual IgG and C3d positivity alone does not prove mixed AIHA; look for a clinically significant cold antibody as well.
Look for an underlying cause selectively
Review recent infections and new medications, and assess for autoimmune disease and lymphoproliferative disorders. Let history, examination and initial laboratory findings guide further tests. The panel advises against routine bone marrow biopsy when there are no clinical or laboratory features suggesting a lymphoproliferative disorder (1D).
Let symptoms and stability guide urgency
Stable patients without symptomatic anaemia may be monitored; acute severe haemolysis may require admission and urgent treatment (1D). If profound anaemia causes end-organ ischaemia or haemodynamic instability, alert the transfusion laboratory immediately. Do not delay a life-saving transfusion for completion of compatibility testing. Follow local emergency-release policy for group O red cells when there is no time to test; when time permits, the laboratory should investigate compatibility and underlying alloantibodies.
Plan steroid taper and escalation together
For symptomatic warm AIHA, the usual initial prednisolone dose is 1 mg/kg/day (1D). Once haemolysis has substantially improved and haemoglobin is stable, start an individualised taper. The panel aims to limit total steroid exposure to three months or less when feasible (1D). An early relapse on taper should prompt second-line treatment rather than simply increasing prednisolone (1C).
The panel recommends rituximab with prednisolone upfront or early when response to corticosteroids is minimal after at least two weeks (1D). The algorithm also flags transfusion dependence as a reason to add it. Persistent disease after front-line treatment warrants specialist reassessment: agents such as mycophenolate and azathioprine can take months to work, so they cannot substitute for urgent rescue treatment. Splenectomy is an option for selected patients, with infection and thrombosis risk central to the decision.
Build supportive care into the plan
The panel recommends folic acid 5 mg daily and thromboprophylaxis during admission for warm AIHA unless contraindicated (both 1D). Assess infection and bone risks during prolonged steroid treatment, including whether Pneumocystis prophylaxis is needed. In pregnancy, involve maternal–fetal medicine and haematology early; fetal anaemia surveillance, neonatal haemoglobin and bilirubin monitoring, and the timing of immunosuppressive treatment need a separate plan.
How to read the recommendations
This is a modified Delphi consensus, developed where high-quality data were limited. In the paper's scheme, 1D denotes a strong recommendation based on expert consensus. The practical aim is to relieve symptomatic anaemia and achieve transfusion independence while limiting treatment toxicity.
Original source: Choi P, Merriman E, Bennett A, et al. AustrAIHANZ consensus guidelines on the investigation and management of autoimmune haemolytic anaemia in Australia and New Zealand. Internal Medicine Journal. First published 8 November 2026. Read the original guideline .