title: Anaplastic large cell lymphoma: an unusual presentation in a 7-year-old girl.
abstract: Anaplastic large cell lymphoma (ALCL) accounts for 10% to 30% of all childhood lymphomas and approximately 5% of all non-Hodgkin's lymphoma. ALCL is considered to be a T-cell non-Hodgkin's lymphoma that can be divided into two major groups with distinct genetic, immunophenotypic, and clinical behaviors. The first group consists of a spectrum of CD30+ T-cell lymphoproliferative disorders that include primary cutaneous ALCL (C-ALCL) and lymphomatoid papulosis. The second group is systemic ALCL (S-ALCL), which is further divided into two subgroups: anaplastic lymphoma kinase positive (ALK+) and ALK-negative. Between 30% and 60% of S-ALCL express ALK, which is usually the result of a t(2;5) translocation that correlates with onset in the first three decades of life, male predominance, and good prognosis. Although morphologically similar, ALK- ALCL shows varied clinical behaviors and immunophenotypes; is commonly seen in older age groups, with a peak incidence in the sixth decade of life with no preference as to sex; and has an overall poorer prognosis. We present a case of CD30+, ALK- S-ALCL in a 7-year-old girl.
authors.affiliation: North Carolina University School of Medicine, Chapel Hill, North Carolina 27516, USA.
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Ju
authors.forename: Chris
authors.initials: C
authors.lastname: Adigun
authors.forename: Cherie
authors.initials: C
authors.lastname: Dunphy
authors.forename: Stuart
authors.initials: S
authors.lastname: Gold
authors.forename: Dean S
authors.initials: DS
authors.lastname: Morrell
mesh_terms: D001365:Axilla; D001706:Biopsy; D002648:Child; D005260:Female; D006801:Humans; D017728:Lymphoma, Large-Cell, Anaplastic / Q000473:pathology*; D012867:Skin / Q000473:pathology*; D012878:Skin Neoplasms / Q000473:pathology*