Sa. Pileri et al., FREQUENT EXPRESSION OF THE NPM-ALK CHIMERIC FUSION PROTEIN IN ANAPLASTIC LARGE-CELL LYMPHOMA, LYMPHO-HISTIOCYTIC TYPE, The American journal of pathology, 150(4), 1997, pp. 1207-1211
The revised European-American lymphoma classification recognizes a sub
type of anaplastic large-cell lymphoma (ALCL), termed lympho-histiocyt
ic 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 histi
ocytes. In this paper, we show that three of five cases of lympbo-hist
iocytic ALCL stain with anti-ALK antibodies and can therefore be presu
med to express the chimeric NPM/ALK protein secondary to (2;5) translo
cation. These findings further support the inclusion of this as a type
of AI;CL and not among the nonanaplastic peripheral T-cell lymphomas.
Furthermore, they indicate that staining for ALK proteins is a powerf
ul tool for the diagnosis of lympho-histiocytic ALCL, the recognition
of which may be difficult on morphological grounds.