Abstract
The clinicopathologic features of anaplastic large cell lymphoma (ALCL) are reviewed. ALCL is a heterogeneous group of tumours, and histologic examination alone is not adequate in providing useful prognostic information. However, using a combination of clinical, phenotypic, and genotypic features, several distinct clinicopathologic entities have been identified. A subset of ALCL as presently defined is characterized by a balanced translocation, t(2;5)(p23;q35), resulting in a novel fusion protein (NPM-ALK) that can be readily detected by immunohistochemical methods using antibodies against the ALK protein. Detection of ALK protein, along with other methods for demonstrating the t(2;5), has assisted in identifying a distinct biologic entity within the heterogeneous group of ALCL with significant prognostic implications. It is important to separate these from cases of ALK-negative ALCL, which have a poorer prognosis, and cases of primary cutaneous ALCL, which have an excellent prognosis.
Copyright 1999 John Wiley & Sons, Ltd.
MeSH terms
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Adolescent
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Adult
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Age of Onset
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Aged
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Antigens, Neoplasm / analysis
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Antineoplastic Combined Chemotherapy Protocols / therapeutic use
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Chromosomes, Human, Pair 2 / genetics
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Chromosomes, Human, Pair 2 / ultrastructure
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Chromosomes, Human, Pair 5 / genetics
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Chromosomes, Human, Pair 5 / ultrastructure
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Doxorubicin / administration & dosage
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Female
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Humans
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Immunophenotyping
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Lymphoma, Large B-Cell, Diffuse / classification
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Lymphoma, Large B-Cell, Diffuse / drug therapy
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Lymphoma, Large B-Cell, Diffuse / epidemiology
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Lymphoma, Large B-Cell, Diffuse / genetics
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Lymphoma, Large B-Cell, Diffuse / pathology*
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Lymphoma, Large B-Cell, Diffuse / radiotherapy
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Male
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Middle Aged
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Neoplasm Proteins / analysis
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Neoplasm Proteins / genetics
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Neoplastic Stem Cells / pathology
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Oncogene Proteins, Fusion / analysis
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Oncogene Proteins, Fusion / genetics
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Prognosis
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Protein-Tyrosine Kinases / analysis
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Protein-Tyrosine Kinases / genetics
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Skin Neoplasms / drug therapy
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Skin Neoplasms / epidemiology
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Skin Neoplasms / genetics
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Skin Neoplasms / pathology
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Skin Neoplasms / radiotherapy
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Translocation, Genetic / genetics
Substances
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Antigens, Neoplasm
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Neoplasm Proteins
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Oncogene Proteins, Fusion
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Doxorubicin
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p80(NPM-ALK) protein
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Protein-Tyrosine Kinases