title: [Management of autoimmune thrombocytopenic purpura: a prospective study of 147 children from the regional network RHémaP].
abstract: OBJECTIVE
  Assessment of the impact of guidelines from a regional pediatric network to standardize the management of childhood immune thrombocytopenic purpura (ITP).
  
  
  METHODS
  Consensus guidelines were drawn up in centers of the pediatric network for hematological diseases, RHémaP, and a cohort of children referred for ITP in these centers was set up. A 1-year follow-up was recorded for each patient over a 43-month period.
  
  
  RESULTS
  We report data from a cohort of 147 children. At diagnosis, we recorded severe thrombocytopenia (median=8G/l) and 141 children had hemorrhagic symptoms (96%). Only 23 children had a bone marrow aspiration (BMA) at diagnosis (16.3%), which meant a high level of implementation of the RHémaP recommendations (96%) since indications of BMA were limited to rare indications. For 135 children (91.8%), treatment fulfilled the RHémaP guidelines that were mainly based on the platelet count: 121 received intraveinous immunoglobulin (IVIG) and 14 were not treated. Among those who received IVIG, 110 were good responders (91%) at the 96-h evaluation (platelet count greater than 20G/l), nine (7.4%) were poor responders, and 1 died of intracranial hemorrhage. At 6 months, chronic ITP was observed in 40 children (32.8%). Chronic ITP was associated with a higher platelet count at diagnosis and an older age (p<10(-3) and p=10(-3), respectively).
  
  
  CONCLUSIONS
  The practices recorded over a 43-month period in our cohort fulfilled the RhémaP guidelines and we conclude that we managed to standardize regional practices for children with ITP. We observed conventional epidemiological characteristics in this cohort. Older children and higher platelet count at diagnosis were significantly associated with higher frequency of chronic ITP.
authors.affiliation: Service de pédiatrie et d'hématologie pédiatrique, hôpital d'enfants la Timone, CHU la Timone, université de la Méditerranée, 13385 Marseille cedex 5, France.
authors.forename: M
authors.initials: M
authors.lastname: Le Meignen
authors.forename: P
authors.initials: P
authors.lastname: Mossler
authors.forename: M
authors.initials: M
authors.lastname: Mates
authors.forename: C
authors.initials: C
authors.lastname: Mathey
authors.forename: A
authors.initials: A
authors.lastname: Hass
authors.forename: F
authors.initials: F
authors.lastname: Monpoux
authors.forename: B
authors.initials: B
authors.lastname: Faverge
authors.forename: G
authors.initials: G
authors.lastname: Fortier
authors.forename: J
authors.initials: J
authors.lastname: Nguyen
authors.forename: L
authors.initials: L
authors.lastname: Belahbib
authors.forename: C
authors.initials: C
authors.lastname: Coache
authors.forename: J-M
authors.initials: JM
authors.lastname: Chamoulli
authors.forename: E
authors.initials: E
authors.lastname: Moulène
authors.forename: N
authors.initials: N
authors.lastname: Sirvent
authors.forename: G
authors.initials: G
authors.lastname: Michel
authors.forename: H
authors.initials: H
authors.lastname: Chambost
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D005602:France; D019983:Guideline Adherence; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D008297:Male; D017410:Practice Guidelines as Topic; D011446:Prospective Studies; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000175:diagnosis* / Q000628:therapy*
chemical_list: D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors