Second-line therapy in paediatric warm autoimmune haemolytic anaemia. Guidelines from the Associazione Italiana Onco-Ematologia Pediatrica (AIEOP)

The present report represents a valuable effort to support Paediatricians with a guide tool for the management of second line therapy of childhood AIHA. To summarize, a second line treatment is indicated in children with AIHA who do not respond to the first line therapy or become steroid dependent. Rituximab is the first choice as second line treatment; in patients resistant to rituximab, cyclophosphamide can be used, before splenectomy. In patients who developed a steroid dependence, an immunosuppressive drug – preferably mycophenolate mofetil, but also azathioprine or cyclosporine – is indicated in order to reduce the dose of the steroid and eventually suspend it. Alentuzumab and HSCT are last choice for refractory, severely symptomatic cases.