Anaplastic Largecell Lymphoma

Previously defined on the basis of its cytology and expression of CD30, it has become clear that more than one "real" disease can exhibit these features. The discovery of the t(2;5) translocation, which results in the expression of anaplastic lymphoma kinase (ALK) protein, has helped to define a form of T/null-cell ALCL that tends to occur in children and young adults and which, although aggressive, carries a good prognosis with appropriate therapy.38 It seems likely that the somewhat similar, but t(2;5) (ALK) negative, cases are a different disorder, but this awaits confirmation. Again, gene profiling is likely to give us the answer. It is clear, however, that primary cutaneous ALCL,39 which is always ALK negative and, moreover, lacks a cytotoxic phenotype, is an entirely different entity.

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