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Anaplastic Large Cell Lymphoma: An Unusual Presentation in a 7‐Year‐Old Girl
Author(s) -
Ju Elizabeth,
Adigun Chris,
Dunphy Cherie,
Gold Stuart,
Morrell Dean S.
Publication year - 2011
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.2011.01465.x
Subject(s) - anaplastic large cell lymphoma , lymphomatoid papulosis , anaplastic lymphoma kinase , cd30 , medicine , lymphoma , large cell , girl , large cell lymphoma , immunophenotyping , pathology , immunology , cancer , lung cancer , biology , adenocarcinoma , antigen , genetics , malignant pleural effusion
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.