Diagnosis and management of newly diagnosed childhood autoimmune haemolytic anaemia. Recommendations from the Red Cell Study Group of the Paediatric Haemato-Oncology Italian Association

Saverio Ladogana, Matteo Maruzzi, Piera Samperi, Silverio Perrotta, Giovanni C Del Vecchio, Lucia D Notarangelo, Piero Farruggia, Federico Verzegnassi, Nicoletta Masera, Paola Saracco, Silvia Fasoli, Maurizio Miano, Gabriella Girelli, Wilma Barcellini, Alberto Zanella, Giovanna Russo, AIHA Committee of the Italian Association of Paediatric Onco-haematology (AIEOP)

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Abstract

Autoimmune haemolytic anaemia is an uncommon disorder to which paediatric haematology centres take a variety of diagnostic and therapeutic approaches. The Red Cell Working Group of the Italian Association of Paediatric Onco-haematology (Associazione Italiana di Ematologia ed Oncologia Pediatrica, AIEOP) developed this document in order to collate expert opinions on the management of newly diagnosed childhood autoimmune haemolytic anaemia.The diagnostic process includes the direct and indirect antiglobulin tests; recommendations are given regarding further diagnostic tests, specifically in the cases that the direct and indirect antiglobulin tests are negative. Clear-cut definitions of clinical response are stated. Specific recommendations for treatment include: dosage of steroid therapy and tapering modality for warm autoimmune haemolytic anaemia; the choice of rituximab as first-line therapy for the rare primary transfusion-dependent cold autoimmune haemolytic anaemia; the indications for supportive therapy; the need for switching to second-line therapy. Each statement is provided with a score expressing the level of appropriateness and the agreement among participants.

Original languageEnglish
Pages (from-to)259-267
Number of pages9
JournalBlood Transfusion
Volume15
Issue number3
DOIs
Publication statusPublished - May 2017

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Ladogana, S., Maruzzi, M., Samperi, P., Perrotta, S., Del Vecchio, G. C., Notarangelo, L. D., Farruggia, P., Verzegnassi, F., Masera, N., Saracco, P., Fasoli, S., Miano, M., Girelli, G., Barcellini, W., Zanella, A., Russo, G., & AIHA Committee of the Italian Association of Paediatric Onco-haematology (AIEOP) (2017). Diagnosis and management of newly diagnosed childhood autoimmune haemolytic anaemia. Recommendations from the Red Cell Study Group of the Paediatric Haemato-Oncology Italian Association. Blood Transfusion, 15(3), 259-267. https://doi.org/10.2450/2016.0072-16