title: Paediatric anaplastic large cell lymphoma with leukaemic presentation in children: a report of nine French cases.
abstract: This study aimed to describe the clinical features and outcome of anaplastic large cell lymphoma (ALCL) with leukaemic presentation in children. Among 267 patients included in the French paediatric ALCL database between 1989 and 2012, nine (3%) were described as having cytologically detectable circulating tumour cells. Clinical features combined fever (8/9), nodal and extra-nodal disease (9/9), including hepato-splenic (9/9) and lung involvement (7/9). The level of hyperleucocytosis ranged from 30 to 120 × 10(9) /l, with 12-90% of tumour cells. Diagnosis relied on a lymph node biopsy, with a positive ALK+ antibody immunostain in all nine cases, a T-cell immunophenotype in 7/9 cases and CD3 positivity in 5/9 cases. A small cell component was present in 6/9 cases. Only four patients achieved a complete remission with first-line therapy and 3/4 relapsed. Four patients are alive with a median follow-up of 31 months, two of them after allogeneic haematopoietic stem cell transplantation (HSCT), and five patients died, two of them of disease. In conclusion, ALCL with leukaemic presentation is very unusual and should be considered as high-risk lymphoma requiring new therapeutic strategies. The respective role of new agents and allogeneic HSCT in first complete remission still has to be assessed.
authors.affiliation: CHU de Strasbourg, Service d'hématologie oncologie pédiatrique, pédiatrie 3, Hopital Hautepierre, Strasbourg, France.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Spiegel
authors.forename: Catherine
authors.initials: C
authors.lastname: Paillard
authors.forename: Stephane
authors.initials: S
authors.lastname: Ducassou
authors.forename: Yves
authors.initials: Y
authors.lastname: Perel
authors.forename: Dominique
authors.initials: D
authors.lastname: Plantaz
authors.forename: Marion
authors.initials: M
authors.lastname: Strullu
authors.forename: Alice
authors.initials: A
authors.lastname: Eischen
authors.forename: Patrick
authors.initials: P
authors.lastname: Lutz
authors.forename: Laurence
authors.initials: L
authors.lastname: Lamant
authors.forename: Marie-Cécile
authors.initials: MC
authors.lastname: Le Deley
authors.forename: Laurence
authors.initials: L
authors.lastname: Brugières
mesh_terms: D000293:Adolescent; D000077548:Anaplastic Lymphoma Kinase; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D002490:Central Nervous System / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D005602:France; D006801:Humans; D007223:Infant; D017730:Ki-1 Antigen / Q000032:analysis; D007938:Leukemia / Q000175:diagnosis; D007964:Leukocytosis / Q000209:etiology*; D008198:Lymph Nodes / Q000473:pathology; D017728:Lymphoma, Large-Cell, Anaplastic / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics; D008297:Male; D014411:Neoplastic Stem Cells / Q000473:pathology; D011505:Protein-Tyrosine Kinases / Q000032:analysis; D020794:Receptor Protein-Tyrosine Kinases / Q000032:analysis; D012008:Recurrence; D012867:Skin / Q000473:pathology; D063189:Symptom Assessment; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor; D017730:Ki-1 Antigen; C107023:p80(NPM-ALK) protein; C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D011505:Protein-Tyrosine Kinases; D020794:Receptor Protein-Tyrosine Kinases
keywords: anaplastic large cell lymphoma; anaplastic lymphoma kinase; leukaemia; paediatric; small cell variant