Management of autoimmune thrombocytopenic purpura: a prospective study of 147 children from the regional network RHémaP

Assessment of the impact of guidelines from a regional pediatric network to standardize the management of childhood immune thrombocytopenic purpura (ITP). Consensus guidelines were drawn up in centers of the pediatric network for hematological diseases, RHémaP, and a cohort of children referred for...

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Published in:Archives de pédiatrie : organe officiel de la Société française de pédiatrie 2008-09, Vol.15 (9), p.1398-1406
Main Authors: Le Meignen, M, Mossler, P, Mates, M, Mathey, C, Hass, A, Monpoux, F, Faverge, B, Fortier, G, Nguyen, J, Belahbib, L, Coache, C, Chamoulli, J-M, Moulène, E, Sirvent, N, Michel, G, Chambost, H
Format: Article
Language:fre
Subjects:
Child
Child, Preschool
Female
France
Guideline Adherence
Humans
Immunoglobulins, Intravenous - therapeutic use
Immunologic Factors - therapeutic use
Male
Practice Guidelines as Topic
Prospective Studies
Purpura, Thrombocytopenic, Idiopathic - diagnosis
Purpura, Thrombocytopenic, Idiopathic - therapy
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Summary:Assessment of the impact of guidelines from a regional pediatric network to standardize the management of childhood immune thrombocytopenic purpura (ITP). 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. 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
ISSN:0929-693X
DOI:10.1016/j.arcped.2008.06.007