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Blood Cells Mol Dis. 2015 Jun;55(1):40-7. doi: 10.1016/j.bcmd.2015.03.007. Epub 2015 Mar 31.

Diagnosis and management of acquired aplastic anemia in childhood. Guidelines from the Marrow Failure Study Group of the Pediatric Haemato-Oncology Italian Association (AIEOP).

Author information

1
Department of Pediatric Onco-Hematology, University Hospital, Parma, Italy.
2
Pediatric Onco-Hematology Unit, Vito Fazzi Hospital, Lecce, Italy.
3
Hematology Unit, Hospital of Pescara, Italy.
4
Institute of Maternal and Child Health IRCCS Burlo Garofalo, Trieste, Italy.
5
Department of Pediatric Hematology, San Gerardo Hospital, Monza, Italy.
6
Pediatric Hematology Oncology Unit, Borgo Roma Hospital, Verona, Italy.
7
Clinical and Experiment Hematology Unit, G. Gaslini Children's Hospital, Genoa, Italy.
8
Hematology and HSCT Unit, University La Sapienza, Rome, Italy.
9
Pediatric Onco-Hematology Unit, Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo, Italy.
10
Department of Pediatric Hematology, S. Camillo Hospital, Rome, Italy.
11
Pediatric Onco-Hematology Unit, A.R.N.A.S. Civico Hospital, Palermo, Italy.
12
Pediatric Onco-Hematology and Transfusional Department, Pugliese Ciaccio Hospital, Catanzaro, Italy.
13
Department of Pediatric Haemato-Oncology, Santobono-Pausilipon Hospital, Naples, Italy.
14
Department of Pediatric, University of Bari, Bari, Italy.
15
Oncology-Haematology and Bone Marrow Transplantation Unit, Children's Hospital, Spedali Civili, Brescia, Italy.
16
Department of Mother and Child, University Hospital of Modena, Modena, Italy.
17
Pediatric Onco-Hematology Unit, University Hospital of Padua, Padua, Italy.
18
University of Turin, Turin, Italy.
19
Division of Pediatric Hematology/Oncology, University of Catania, Catania, Italy.
20
Pediatric Onco-Hematology, Regina Margherita Children's Hospital, Turin, Italy.
21
Department of Pediatric Onco-Hematology, Meyer Children's Hospital, Florence, Italy.
22
Pediatric Hematology/Oncology Department, IRCCS San Matteo Hospital, Pavia, Italy.
23
Clinical and Experiment Hematology Unit, G. Gaslini Children's Hospital, Genoa, Italy. Electronic address: carlodufour@ospedale-gaslini.ge.it.

Abstract

Acquired aplastic anemia (AA) is a rare heterogeneous disease characterized by pancytopenia and hypoplastic bone marrow. The incidence is 2-3/million inhabitants/year, in Europe, but higher in East Asia. Survival in severe aplastic anemia (SAA) has markedly improved in the past 2 decades because of advances in hematopoietic stem cell transplantation, immunosuppressive and biologic drugs, and supportive care. In SAA hematopoietic stem cell transplant (HSCT) from a matched sibling donor (MSD) is the treatment of choice. If a MSD is not available, the options include immunosuppressive therapy (IST) or unrelated donor HSCT. The objective of this guideline is to provide healthcare professionals with clear guidance on the diagnosis and management of pediatric patients with AA. A preliminary, evidence-based document issued by a group of pediatric hematologists was discussed, modified and approved during a series of "Consensus Conferences" according to procedures previously validated by the AIEOP Board. The guidelines highlight the importance of referring pediatric patients with AA to pediatric centers with long experience in diagnosis, differential diagnosis, management, supportive care and follow-up of AA.

KEYWORDS:

Acquired aplastic anemia; Childhood; Guidelines

PMID:
25976466
DOI:
10.1016/j.bcmd.2015.03.007
[Indexed for MEDLINE]

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