Recommendations for the management of acute immune thrombocytopenia in children. A Consensus Conference from the Italian Association of Pediatric Hematology and Oncology.

Journal

Blood transfusion = Trasfusione del sangue
ISSN: 2385-2070
Titre abrégé: Blood Transfus
Pays: Italy
ID NLM: 101237479

Informations de publication

Date de publication:
27 Jul 2023
Historique:
received: 11 03 2023
accepted: 07 06 2023
medline: 7 9 2023
pubmed: 7 9 2023
entrez: 7 9 2023
Statut: aheadofprint

Résumé

Immune thrombocytopenia (ITP) is an acquired immune-mediated bleeding disorder characterized by isolated thrombocytopenia. Its estimated yearly incidence in the pediatric population is 1.9-6.4/100,000. ITP in children is usually a self-limiting and benign disorder. The clinical management of children with ITP often remains controversial, as robust randomized trials on the management of this disorder are lacking. Treatments vary widely in clinical practice and existing guidelines from hematology societies on clinical management offer indications based largely on expert opinion rather than strong evidence. The Coagulative Disorder Working Group of the Italian Association of Pediatric Hematology and Oncology (AIEOP) developed this document to collect shared expert opinions on the management of newly diagnosed ITP, updating previous guidelines and providing recommendations to pediatricians. Each statement has been given a score expressing the strength of evidence, appropriateness and agreement among participants. Clear-cut definitions of the clinical phases of the disease and clinical response are stated. Recommendations are given regarding the classification of bleeding symptoms, evaluation of bleeding risk, diagnosis, and prognostic factors. Specific recommendations for treatment include indications for first-line (intravenous immunoglobulins, steroids) and second-line (combined therapy, thrombopoietin receptor agonists, immunosuppressive drugs, rituximab) therapeutic agents, as well as hemorrhagic emergency and supportive treatment, including emergency splenectomy. The optimal follow-up schedule, the relation between ITP and vaccines and health-related quality-of-life issues are also discussed. The panel achieved broad consensus on issues related to how to treat children with newly diagnosed ITP, providing a comprehensive review of all relevant clinical aspects.

Sections du résumé

BACKGROUND BACKGROUND
Immune thrombocytopenia (ITP) is an acquired immune-mediated bleeding disorder characterized by isolated thrombocytopenia. Its estimated yearly incidence in the pediatric population is 1.9-6.4/100,000. ITP in children is usually a self-limiting and benign disorder. The clinical management of children with ITP often remains controversial, as robust randomized trials on the management of this disorder are lacking. Treatments vary widely in clinical practice and existing guidelines from hematology societies on clinical management offer indications based largely on expert opinion rather than strong evidence.
MATERIALS AND METHODS METHODS
The Coagulative Disorder Working Group of the Italian Association of Pediatric Hematology and Oncology (AIEOP) developed this document to collect shared expert opinions on the management of newly diagnosed ITP, updating previous guidelines and providing recommendations to pediatricians. Each statement has been given a score expressing the strength of evidence, appropriateness and agreement among participants.
RESULTS RESULTS
Clear-cut definitions of the clinical phases of the disease and clinical response are stated. Recommendations are given regarding the classification of bleeding symptoms, evaluation of bleeding risk, diagnosis, and prognostic factors. Specific recommendations for treatment include indications for first-line (intravenous immunoglobulins, steroids) and second-line (combined therapy, thrombopoietin receptor agonists, immunosuppressive drugs, rituximab) therapeutic agents, as well as hemorrhagic emergency and supportive treatment, including emergency splenectomy. The optimal follow-up schedule, the relation between ITP and vaccines and health-related quality-of-life issues are also discussed.
DISCUSSION CONCLUSIONS
The panel achieved broad consensus on issues related to how to treat children with newly diagnosed ITP, providing a comprehensive review of all relevant clinical aspects.

Identifiants

DOI: 10.2450/BloodTransfus.501 PMID: 37677093
pubmed: 37677093
pii: BloodTransfus.501
doi: 10.2450/BloodTransfus.501
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Auteurs

Giovanna Russo (G)

Pediatric Onco-hematology Unit, Azienda Policlinico Rodolico San Marco, Department of Clinical and Experimental Medicine, University of Catania, Italy.

Emilia Parodi (E)

Pediatric and Neonatology Unit, Ordine Mauriziano Hospital, Turin, Italy.

Piero Farruggia (P)

Pediatric Hematology and Oncology Unit, ARNAS Ospedale Civico, Palermo, Italy.

Lucia D Notarangelo (LD)

Direzione Medica di Presidio, Children's Hospital, ASST-Spedali Civili, Brescia, Italy.

Silverio Perrotta (S)

Department of Women, Children and General and Specialized Surgery, "Luigi Vanvitelli" Università degli Studi della Campania, Naples, Italy.

Maddalena Casale (M)

Department of Women, Children and General and Specialized Surgery, "Luigi Vanvitelli" Università degli Studi della Campania, Naples, Italy.

Simone Cesaro (S)

Pediatric Hematology Oncology, Department of Mother and Child, Azienda Ospedaliera Universitaria Integrata Verona, Verona, Italy.

Giovanni Del Borrello (G)

Pediatric Oncohematology, Pediatrics Department, Hospital Città Della Salute e Della Scienza, University of Turin, Turin, Italy.

Giovanni C Del Vecchio (GC)

Interdisciplinary Department of Medicine, Pediatric Section, "Aldo Moro" University of Bari, Bari, Italy.

Fiorina Giona (F)

Department of Translational and Precision Medicine, Sapienza University of Rome, AOU Policlinico Umberto I, Rome, Italy.

Chiara Gorio (C)

Pediatric Onco-hematology Unit, Children's Hospital, ASST-Spedali Civili, Brescia, Italy.

Saverio Ladogana (S)

Pediatric Onco-hematology Unit "Casa Sollievo della Sofferenza" Hospital, IRCCS, San Giovanni Rotondo, Italy.

Giuseppe Lassandro (G)

Interdisciplinary Department of Medicine, Pediatric Section, "Aldo Moro" University of Bari, Bari, Italy.

Antonio Marzollo (A)

Pediatric Hematology, Oncology and Stem Cell Transplant Division, Padua University Hospital, Padua, Italy.

Karolina Maslak (K)

Pediatric Onco-hematology Unit, Azienda Policlinico Rodolico San Marco, Department of Clinical and Experimental Medicine, University of Catania, Italy.

Maurizio Miano (M)

Hematology Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy.

Margherita Nardi (M)

Pediatric Hematology Oncology, Bone Marrow Transplant, Azienda Ospedaliero Universitaria Pisana, S. Chiara Hospital, Pisa, Italy.

Giuseppe Palumbo (G)

Department of Pediatric Hematology and Oncology Cell and Gene Therapy, Bambino Gesù Children's Hospital, IRCCS, Department of Systems Medicine, University of Tor Vergata, Rome, Italy.

Francesca Rossi (F)

Department of Women, Children and General and Specialized Surgery, "Luigi Vanvitelli" Università degli Studi della Campania, Naples, Italy.

Marco Spinelli (M)

Pediatric Hematology Oncology Unit, Department of Pediatrics, IRCCS San Gerardo dei Tintori Foundation, Monza, Italy.

Alessandra Tolva (A)

Pediatric Hematology-Oncology, IRCCS Policlinico San Matteo, Pavia, Italy.

Paola Saracco (P)

Department of Public Health and Pediatric Sciences, Regina Margherita Children's Hospital, University of Turin, Turin, Italy.

Ugo Ramenghi (U)

Department of Public Health and Pediatric Sciences, Regina Margherita Children's Hospital, University of Turin, Turin, Italy.

Paola Giordano (P)

Interdisciplinary Department of Medicine, Pediatric Section, "Aldo Moro" University of Bari, Bari, Italy.

Classifications MeSH