The diagnosis, prognosis, and management of autoimmune hemolytic anemia (AIHA) continue to be challenging in current practice. This is related to an incomplete understanding of the pathophysiology of the disease process, complexity of initiating factors, and a lack of evidencebased standardized therapies. There is no completely validated and standard therapeutic approach to AIHA, because randomized clinical trials are difficult to implement.
The objective of this guideline is to provide healthcare professionals with guidance on the management of patients with secondary autoimmune haemolytic anaemia (AIHA). The guidance may not be appropriate to every patient and in all cases individual patient circumstances may dictate an alternative approach.
Since 2004, a national observational study has been aiming to thoroughly describe cases and identify prognostic factors. Patients from all French hematologic pediatric units have been included if they had a hemoglobin concentration less than 11 g/dL, a positive direct antiglobulin test and hemolysis. Evans’ syndrome was defined by the association of autoimmune hemolytic anemia and immunological thrombocytopenic purpura. Data from patients' medical records were registered from birth to last follow-up. Autoimmune hemolytic anemia was classified as primary or secondary. Remission criteria, qualifying the status of anemia at last follow-up, were used with the aim of identifying a subgroup with a favorable prognosis in continuous complete remission.