Warm Autoimmune Hemolytic Anemia: Treatment

Warm Autoimmune Hemolytic Anemia: overview

This algorithm outlines treatment of warm autoimmune hemolytic anemia, defined by a positive direct antiglobulin test for IgG, whether idiopathic or secondary to an underlying condition. The chart is organized by line of therapy.

First-line treatment is corticosteroids, typically prednisone with a subsequent taper. Rituximab may be added early as a steroid-sparing agent. In the second line, rituximab is used if it was not given up front, and splenectomy is the surgical option, producing durable responses in a subset of patients. For refractory disease, the chart lists steroid-sparing immunosuppressants: azathioprine, mycophenolate mofetil (MMF) or cyclophosphamide. The overall sequence moves from corticosteroids, to anti-CD20 therapy, to splenectomy or broader immunosuppression as disease proves resistant.

Trials behind this algorithm

Birgens 2013 (Rituximab + prednisolone vs prednisolone, warm AIHA) Birgens H et al, Br J Haematol, 2013; PMID: 23981017

Background reading: Hemolytic anemias board review notes.

Warm Autoimmune Hemolytic Anemia: the algorithm in words

Starting point: Warm AIHA DAT IgG+ (idiopathic / secondary). Full background: Hemolytic anemias notes.

First-line (first-line): Corticosteroids (prednisone ± taper); Add early: Rituximab (with steroids · Birgens 2013).
Second-line (second-line): Anti-CD20: Rituximab (if not used); Surgical: Splenectomy (durable in subset).
Refractory (later-line): Immunosuppress: Azathioprine / MMF / cyclophosphamide (steroid-sparing).