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Clinical analysis and prognostic significance of haemophagocytic lymphohistiocytosis‐associated anaplastic large cell lymphoma in children
Author(s) -
Pasqualini Claudia,
MinardColin Veronique,
Saada Veronique,
Lamant Laurence,
Delsol Georges,
Patte Catherine,
Deley MarieCécile,
ValteauCouanet Dominique,
Brugières Laurence
Publication year - 2014
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.12728
Subject(s) - hemophagocytic lymphohistiocytosis , medicine , anaplastic large cell lymphoma , incidence (geometry) , lymphoma , bone marrow , histiocyte , gastroenterology , pathology , disease , physics , optics
Summary 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 .