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Blood Transfus. 2017 May;15(3):259-267. doi: 10.2450/2016.0072-16. Epub 2016 Dec 16.

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

Author information

1
Paediatric Onco-haematology Unit, "Casa Sollievo della Sofferenza" Hospital, IRCCS, San Giovanni Rotondo, Italy.
2
Paediatric Onco-haematology Unit, Azienda Policlinico "Vittorio Emanuele", Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy.
3
Department of Women, Children and General and Specialized Surgery, Second University of Naples, Naples, Italy.
4
"F. Vecchio" Paediatric Unit, University Hospital, Policlinico Hospital, Bari, Italy.
5
Paediatric Onco-haematology Unit, Children's Hospital, Spedali Civili, Brescia, Italy.
6
Paediatric Onco-haematology Unit, Civico Hospital, Palermo, Italy.
7
Paediatric Onco-haematology Unit, "Burlo Garofolo" Hospital, Trieste, Italy.
8
Paediatric Department, University of Milano-Bicocca, "San Gerardo" Hospital, Monza, Italy.
9
Paediatric and Adolescent Science Department, University of Turin, Turin, Italy.
10
Paediatric Unit, "Carlo Poma" Hospital, Mantua, Italy.
11
Clinical and Experimental Haematology Unit, "G. Gaslini" Children's Hospital, Genoa, Italy.
12
Immunohaematology and Transfusion Medicine Unit, Policlinico Umberto I, Sapienza University, Rome, Italy.
13
Onco-haematology, Physiopathology of Anaemia Unit, IRCCS Ca' Granda Foundation, Ospedale Maggiore Policlinico, Milan, Italy.

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.

PMID:
28151390
PMCID:
PMC5448833
DOI:
10.2450/2016.0072-16
[Indexed for MEDLINE]
Free PMC Article

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