title: Anaplastic large cell lymphomas: a study of 75 pediatric patients.
abstract: In this article, we describe the morphologic and immunophenotypic features of 75 cases of pediatric anaplastic large cell lymphoma (ALCL). According to the World Health Organization classification, 49 cases were common subtype ALCL, and respectively, 3, 6, and 17 cases were small cell, lymphohistiocytic, or mixed histologic variants. Anaplastic lymphoma kinase positivity was detected in 90.7% of the tumors and, using a panel of 9 T-cell surface markers, 88% could be assigned to the T-cell lineage. A molecular analysis for the T-cell receptor gamma (TCR- gamma) and the heavy chain of the immunoglobulin H rearrangements was performed on 6/9 ALCLs with a null immunophenotype, and a TCR clonal pattern was detected in 5/6 cases. In addition, 94.1% were immunoreactive for 1 or more cytotoxic proteins (Tia1, granzyme B, or perforin), and 15% expressed CD56. Clusterin, CD83, and Pax5, respectively, expressed in 91.3%, 1.7%, and 0% of the ALCLs, were useful biomarkers for the differential diagnosis with Hodgkin's lymphomas.
authors.affiliation: Department of Pathology, Ospedale San Bortolo, Vicenza, Italy. emanuele.damore@unipd.it
authors.forename: E S G
authors.initials: ES
authors.lastname: d'Amore
authors.forename: A
authors.initials: A
authors.lastname: Menin
authors.forename: E
authors.initials: E
authors.lastname: Bonoldi
authors.forename: P
authors.initials: P
authors.lastname: Bevilacqua
authors.forename: S
authors.initials: S
authors.lastname: Cazzavillan
authors.forename: V
authors.initials: V
authors.lastname: Donofrio
authors.forename: C
authors.initials: C
authors.lastname: Gambini
authors.forename: M
authors.initials: M
authors.lastname: Forni
authors.forename: A
authors.initials: A
authors.lastname: Gentile
authors.forename: G
authors.initials: G
authors.lastname: Magro
authors.forename: R
authors.initials: R
authors.lastname: Boldrini
authors.forename: M
authors.initials: M
authors.lastname: Pillon
authors.forename: A
authors.initials: A
authors.lastname: Rosolen
authors.forename: R
authors.initials: R
authors.lastname: Alaggio
mesh_terms: D015703:Antigens, CD / Q000276:immunology*; D014408:Biomarkers, Tumor / Q000276:immunology*; D019002:CD56 Antigen / Q000276:immunology*; D002648:Child; D051152:Clusterin / Q000276:immunology*; D003937:Diagnosis, Differential; D005260:Female; D053804:Granzymes / Q000276:immunology; D006689:Hodgkin Disease / Q000175:diagnosis / Q000276:immunology / Q000473:pathology; D006801:Humans; D007136:Immunoglobulins / Q000276:immunology*; D007150:Immunohistochemistry; D016130:Immunophenotyping; D008215:Lymphocytes, Null / Q000276:immunology / Q000473:pathology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis / Q000276:immunology* / Q000473:pathology; D017728:Lymphoma, Large-Cell, Anaplastic / Q000276:immunology / Q000473:pathology; D008297:Male; D008562:Membrane Glycoproteins / Q000276:immunology*; D051757:PAX5 Transcription Factor / Q000276:immunology*; D054353:Perforin; D039102:Poly(A)-Binding Proteins / Q000276:immunology; D052899:Pore Forming Cytotoxic Proteins / Q000276:immunology; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000276:immunology; D000074019:T-Cell Intracellular Antigen-1; D000097802:CD83 Antigen
chemical_list: D015703:Antigens, CD; D014408:Biomarkers, Tumor; D019002:CD56 Antigen; D051152:Clusterin; D053804:Granzymes; D007136:Immunoglobulins; D008562:Membrane Glycoproteins; D051757:PAX5 Transcription Factor; D054353:Perforin; D039102:Poly(A)-Binding Proteins; D052899:Pore Forming Cytotoxic Proteins; D016692:Receptors, Antigen, T-Cell, gamma-delta; D000074019:T-Cell Intracellular Antigen-1; D000097802:CD83 Antigen; C495242:CLU protein, human; C497281:PAX5 protein, human; C071558:TIA1 protein, human