title: Clinical analysis and prognostic significance of haemophagocytic lymphohistiocytosis-associated anaplastic large cell lymphoma in children.
abstract: Haemophagocytic lymphohistiocytosis (HLH) has been rarely described in children treated for an anaplastic large-cell lymphoma (ALCL). We evaluated the incidence, the clinical and histological characteristics and the prognosis of HLH associated-ALCL. The medical, biological, cytological and histological data of patients treated for ALK-positive ALCL in the paediatric department of a single institution between 1975 and 2008 were analysed and assessed for HLH according to diagnosis criteria of the Histiocyte Society. Data concerning a series of 50 consecutive children with ALCL were reviewed. HLH-associated ALCL was observed in 12% of the patients. Lung involvement was significantly more frequent in HLH-associated ALCL patients than in the group without HLH (P = 0·004), as well as central nervous system (CNS) and bone marrow involvement (P = 0·001 and P = 0·007 respectively). The histological subtype in children with HLH-associated ALCL did not differ from that of the group without HLH. There was no significant difference between the two groups in 5-year EFS and OS (P = 0·91 and P > 0·99 respectively). In conclusion, HLH is not rare in paediatric ALCL. Despite a high incidence of visceral, CNS and bone marrow involvement, HLH does not seem to exert a significant impact on outcome in children treated for ALCL.
authors.affiliation: Department of Paediatric Oncology, Institut Gustave Roussy, Villejuif, France.
authors.forename: Claudia
authors.initials: C
authors.lastname: Pasqualini
authors.forename: Veronique
authors.initials: V
authors.lastname: Minard-Colin
authors.forename: Veronique
authors.initials: V
authors.lastname: Saada
authors.forename: Laurence
authors.initials: L
authors.lastname: Lamant
authors.forename: Georges
authors.initials: G
authors.lastname: Delsol
authors.forename: Catherine
authors.initials: C
authors.lastname: Patte
authors.forename: Marie-Cécile
authors.initials: MC
authors.lastname: Le Deley
authors.forename: Dominique
authors.initials: D
authors.lastname: Valteau-Couanet
authors.forename: Laurence
authors.initials: L
authors.lastname: Brugières
keywords: anaplastic large cell lymphoma; child; haemophagocytic lymphohistiocytosis; haemophagocytosis; perforin