Anaplastic large cell lymphoma: one or more entities among T-cell lymphoma? 2009

Alessandro Fornari, and Roberto Piva, and Roberto Chiarle, and Domenico Novero, and Giorgio Inghirami
Department of Pathology and Center for Experimental Research and Medical Studies (CeRMS), University of Torino, Italy. alefornari77@gmail.com

Anaplastic large cell lymphoma (ALCL) is a subtype of peripheral T-cell lymphoma (PTCL) first described in 1985 as a lymphoid malignancy characterized by marked cellular pleomorphism, propensity to grow cohesively, tendency to invade lymph node sinuses and diffuse expression of CD30 1. The discovery of the t(2;5), involving the anaplastic lymphoma kinase (ALK) gene on chromosome 2 and the nucleophosmin (NPM) gene on chromosome 5 in the majority of systemic ALCL, has soon pointed out that ALCL is a clinically and biologically heterogeneous disease. While ALK-positive (ALK+) ALCL is usually characterized by onset in children and young adults and better prognosis, epidemiology, poor outcome and possibly genetic defects of ALK-negative (ALK-) ALCL suggest that this neoplasms should be considered an independent pathological entity. The aim of this review is to illustrate clinical features, histology, immunophenotype, genetics and biology of ALCL and discuss possible relationship(s) among different T-non-Hodgkin lymphoma (T-NHL).

UI MeSH Term Description Entries
D011505 Protein-Tyrosine Kinases Protein kinases that catalyze the PHOSPHORYLATION of TYROSINE residues in proteins with ATP or other nucleotides as phosphate donors. Tyrosine Protein Kinase,Tyrosine-Specific Protein Kinase,Protein-Tyrosine Kinase,Tyrosine Kinase,Tyrosine Protein Kinases,Tyrosine-Specific Protein Kinases,Tyrosylprotein Kinase,Kinase, Protein-Tyrosine,Kinase, Tyrosine,Kinase, Tyrosine Protein,Kinase, Tyrosine-Specific Protein,Kinase, Tyrosylprotein,Kinases, Protein-Tyrosine,Kinases, Tyrosine Protein,Kinases, Tyrosine-Specific Protein,Protein Kinase, Tyrosine-Specific,Protein Kinases, Tyrosine,Protein Kinases, Tyrosine-Specific,Protein Tyrosine Kinase,Protein Tyrosine Kinases,Tyrosine Specific Protein Kinase,Tyrosine Specific Protein Kinases
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000077548 Anaplastic Lymphoma Kinase A receptor tyrosine kinase that is essential for development and differentiation of the nervous system in response to secreted growth factors. It phosphorylates the first tyrosine of the Y-x-x-x-Y-Y motif of targets that include PROTO-ONCOGENE PROTEINS C-CBL; INSULIN RECEPTOR SUBSTRATE-1; and MITOGEN-ACTIVATED PROTEIN KINASES, leading to activation of the MAPK signaling pathway and cell proliferation. A chromosomal aberration involving the ALK gene results in its constitutive expression in some cases of NON-HODGKIN LYMPHOMA. ALK Kinase,ALK Tyrosine Kinase Receptor,Anaplastic Lymphoma Receptor Tyrosine Kinase,CD246 Antigen,NPM-ALK,Nucleophosmin-Anaplastic Lymphoma Kinase,Nucleophosmin Anaplastic Lymphoma Kinase
D016411 Lymphoma, T-Cell, Peripheral A group of malignant lymphomas thought to derive from peripheral T-lymphocytes in lymph nodes and other nonlymphoid sites. They include a broad spectrum of lymphocyte morphology, but in all instances express T-cell markers admixed with epithelioid histiocytes, plasma cells, and eosinophils. Although markedly similar to large-cell immunoblastic lymphoma (LYMPHOMA, LARGE-CELL, IMMUNOBLASTIC), this group's unique features warrant separate treatment. Peripheral T-Cell Lymphoma,T-Cell Lymphoma, Peripheral,Lymphoma, T Cell, Peripheral,Lymphoma, Peripheral T-Cell,Lymphomas, Peripheral T-Cell,Peripheral T Cell Lymphoma,Peripheral T-Cell Lymphomas,T Cell Lymphoma, Peripheral,T-Cell Lymphomas, Peripheral
D017728 Lymphoma, Large-Cell, Anaplastic A systemic, large-cell, non-Hodgkin, malignant lymphoma characterized by cells with pleomorphic appearance and expressing the CD30 ANTIGEN. These so-called "hallmark" cells have lobulated and indented nuclei. This lymphoma is often mistaken for metastatic carcinoma and MALIGNANT HISTIOCYTOSIS. Anaplastic Large-Cell Lymphoma,CD30-Positive Anaplastic Large-Cell Lymphoma,Lymphoma, Large-Cell, Ki-1,CD30+ Anaplastic Large-Cell Lymphoma,Ki-1 Lymphoma,Systemic Anaplastic Large-Cell Lymphoma,Anaplastic Large Cell Lymphoma,Anaplastic Large-Cell Lymphomas,CD30 Positive Anaplastic Large Cell Lymphoma,CD30+ Anaplastic Large Cell Lymphoma,Ki 1 Lymphoma,Ki-1 Lymphomas,Large-Cell Lymphoma, Anaplastic,Large-Cell Lymphomas, Anaplastic,Lymphoma, Anaplastic Large-Cell,Lymphoma, Ki-1,Lymphomas, Anaplastic Large-Cell,Lymphomas, Ki-1,Systemic Anaplastic Large Cell Lymphoma
D020794 Receptor Protein-Tyrosine Kinases A class of cellular receptors that have an intrinsic PROTEIN-TYROSINE KINASE activity. PTK Receptor,Receptors, Protein-Tyrosine Kinase,Tyrosine Kinase Linked Receptor,Tyrosine Kinase Linked Receptors,Tyrosine Kinase Receptor,Tyrosine Kinase Receptors,PTK Receptors,Protein-Tyrosine Kinase Receptor,Receptor Protein-Tyrosine Kinase,Kinase Receptor, Tyrosine,Kinase, Receptor Protein-Tyrosine,Kinases, Receptor Protein-Tyrosine,Protein-Tyrosine Kinase Receptors,Protein-Tyrosine Kinase, Receptor,Protein-Tyrosine Kinases, Receptor,Receptor Protein Tyrosine Kinase,Receptor Protein Tyrosine Kinases,Receptor, PTK,Receptor, Protein-Tyrosine Kinase,Receptor, Tyrosine Kinase,Receptors, PTK,Receptors, Protein Tyrosine Kinase

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