title: Frequent expression of the NPM-ALK chimeric fusion protein in anaplastic large-cell lymphoma, lympho-histiocytic type.
abstract: The revised European-American lymphoma classification recognizes a subtype of anaplastic large-cell lymphoma (ALCL), termed lympho-histiocytic because of its peculiar cytological composition. As in the case of classical ALCL, this tumor usually occurs in young patients and shows an excellent response to chemotherapy, but some authors have suggested that in reality this is a nonanaplastic T-cell lymphoma rich in histiocytes. In this paper, we show that three of five cases of lympho-histiocytic ALCL stain with anti-ALK antibodies and can therefore be presumed to express the chimeric NPM/ALK protein secondary to (2;5) translocation. These findings further support the inclusion of this as a type of ALCL and not among the nonanaplastic peripheral T-cell lymphomas. Furthermore, they indicate that staining for ALK proteins is a powerful tool for the diagnosis of lympho-histiocytic ALCL, the recognition of which may be difficult on morphological grounds.
authors.affiliation: Service of Pathologic Anatomy and Hematopathology Section, Bologna University, Italy.
authors.forename: S A
authors.initials: SA
authors.lastname: Pileri
authors.forename: K
authors.initials: K
authors.lastname: Pulford
authors.forename: S
authors.initials: S
authors.lastname: Mori
authors.forename: D Y
authors.initials: DY
authors.lastname: Mason
authors.forename: E
authors.initials: E
authors.lastname: Sabattini
authors.forename: G
authors.initials: G
authors.lastname: Roncador
authors.forename: M
authors.initials: M
authors.lastname: Piccioli
authors.forename: C
authors.initials: C
authors.lastname: Ceccarelli
authors.forename: P P
authors.initials: PP
authors.lastname: Piccaluga
authors.forename: D
authors.initials: D
authors.lastname: Santini
authors.forename: O
authors.initials: O
authors.lastname: Leone
authors.forename: H
authors.initials: H
authors.lastname: Stein
authors.forename: B
authors.initials: B
authors.lastname: Falini
mesh_terms: D000293:Adolescent; D000077548:Anaplastic Lymphoma Kinase; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D006801:Humans; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis / Q000235:genetics* / Q000378:metabolism; D008228:Lymphoma, Non-Hodgkin / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D009363:Neoplasm Proteins / Q000096:biosynthesis* / Q000235:genetics*; D009687:Nuclear Proteins / Q000096:biosynthesis* / Q000235:genetics*; D000090243:Nucleophosmin; D010750:Phosphoproteins / Q000096:biosynthesis / Q000235:genetics; D011505:Protein-Tyrosine Kinases / Q000096:biosynthesis* / Q000235:genetics*; D020794:Receptor Protein-Tyrosine Kinases; D014178:Translocation, Genetic
chemical_list: D009363:Neoplasm Proteins; D009687:Nuclear Proteins; D010750:Phosphoproteins; D000090243:Nucleophosmin; C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D011505:Protein-Tyrosine Kinases; D020794:Receptor Protein-Tyrosine Kinases