Diagnosis and management of newly diagnosed childhood autoimmune haemolytic anaemia. Recommendations from the Red Cell Study Group of the Paediatric Haemato-Oncology Italian Association.
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.
Paediatric Onco-haematology Unit, "Casa Sollievo della Sofferenza" Hospital, IRCCS, San Giovanni Rotondo, Italy.
Saverio
S
Ladogana
Paediatric Onco-haematology Unit, "Casa Sollievo della Sofferenza" Hospital, IRCCS, San Giovanni Rotondo, Italy.
Matteo
M
Maruzzi
Paediatric Onco-haematology Unit, Azienda Policlinico "Vittorio Emanuele", Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy.
Piera
P
Samperi
Department of Women, Children and General and Specialized Surgery, Second University of Naples, Naples, Italy.
Silverio
S
Perrotta
"F. Vecchio" Paediatric Unit, University Hospital, Policlinico Hospital, Bari, Italy.
Giovanni C
GC
Del Vecchio
Paediatric Onco-haematology Unit, Children's Hospital, Spedali Civili, Brescia, Italy.
Lucia D
LD
Notarangelo
Paediatric Onco-haematology Unit, Civico Hospital, Palermo, Italy.
Piero
P
Farruggia
Paediatric Onco-haematology Unit, "Burlo Garofolo" Hospital, Trieste, Italy.
Federico
F
Verzegnassi
Paediatric Department, University of Milano-Bicocca, "San Gerardo" Hospital, Monza, Italy.
Nicoletta
N
Masera
Paediatric and Adolescent Science Department, University of Turin, Turin, Italy.
Paola
P
Saracco
Paediatric Unit, "Carlo Poma" Hospital, Mantua, Italy.
Silvia
S
Fasoli
Clinical and Experimental Haematology Unit, "G. Gaslini" Children's Hospital, Genoa, Italy.
Maurizio
M
Miano
Immunohaematology and Transfusion Medicine Unit, Policlinico Umberto I, Sapienza University, Rome, Italy.
Gabriella
G
Girelli
Onco-haematology, Physiopathology of Anaemia Unit, IRCCS Ca' Granda Foundation, Ospedale Maggiore Policlinico, Milan, Italy.
Wilma
W
Barcellini
Onco-haematology, Physiopathology of Anaemia Unit, IRCCS Ca' Granda Foundation, Ospedale Maggiore Policlinico, Milan, Italy.
Alberto
A
Zanella
Paediatric Onco-haematology Unit, Azienda Policlinico "Vittorio Emanuele", Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy.
Giovanna
G
Russo
D000744:Anemia, Hemolytic, Autoimmune / Q000139:chemically induced / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D001803:Blood Transfusion / Q000379:methods; D002648:Child; D003298:Coombs Test / Q000379:methods; D019468:Disease Management; D006405:Hematology / Q000379:methods; D006801:Humans; D007075:Immunoglobulin M / Q000032:analysis; D007558:Italy / Q000453:epidemiology; D010372:Pediatrics / Q000379:methods; D012955:Societies, Medical; D013256:Steroids / Q000627:therapeutic use
D007075:Immunoglobulin M; D013256:Steroids