Anaplastic large cell lymphomas: a study of 75 pediatric patients

Pediatr Dev Pathol. May-Jun 2007;10(3):181-91. doi: 10.2350/06-04-0082.1.

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.

Publication types

  • Comparative Study

MeSH terms

  • Antigens, CD / immunology*
  • Biomarkers, Tumor / immunology*
  • CD56 Antigen / immunology*
  • Child
  • Clusterin / immunology*
  • Diagnosis, Differential
  • Female
  • Granzymes / immunology
  • Hodgkin Disease / diagnosis
  • Hodgkin Disease / immunology
  • Hodgkin Disease / pathology
  • Humans
  • Immunoglobulins / immunology*
  • Immunohistochemistry
  • Immunophenotyping
  • Lymphocytes, Null / immunology
  • Lymphocytes, Null / pathology
  • Lymphoma, Large B-Cell, Diffuse / diagnosis
  • Lymphoma, Large B-Cell, Diffuse / immunology*
  • Lymphoma, Large B-Cell, Diffuse / pathology
  • Lymphoma, Large-Cell, Anaplastic / immunology
  • Lymphoma, Large-Cell, Anaplastic / pathology
  • Male
  • Membrane Glycoproteins / immunology*
  • PAX5 Transcription Factor / immunology*
  • Perforin
  • Poly(A)-Binding Proteins / immunology
  • Pore Forming Cytotoxic Proteins / immunology
  • Receptors, Antigen, T-Cell, gamma-delta / immunology
  • T-Cell Intracellular Antigen-1

Substances

  • Antigens, CD
  • Biomarkers, Tumor
  • CD56 Antigen
  • CD83 antigen
  • CLU protein, human
  • Clusterin
  • Immunoglobulins
  • Membrane Glycoproteins
  • PAX5 Transcription Factor
  • PAX5 protein, human
  • Poly(A)-Binding Proteins
  • Pore Forming Cytotoxic Proteins
  • Receptors, Antigen, T-Cell, gamma-delta
  • T-Cell Intracellular Antigen-1
  • TIA1 protein, human
  • Perforin
  • Granzymes