Prognostic significance of anaplastic lymphoma kinase (ALK) protein expression in adults with anaplastic large cell lymphoma

摘要: Anaplastic large cell lymphoma (ALCL) is an aggressive that frequently associated with the t(2;5)(p23;q35), resulting in expression of a fusion protein, nucleophosmin-anaplastic kinase (NPM-ALK), which can be detected by either monoclonal or polyclonal antibodies to ALK protein. The clinical features adults ALCL are incompletely described, and prognostic factors useful for predicting survival remain unclear. This report describes laboratory findings 70 systemic who were treated curative intent. We attempted identify pathological importance, including International Prognostic Index (IPI), immunophenotype, median age patients was 49 years (range, 15 75). There 26 women 44 men follow-up 50 months living patients. Advanced stage present 56% B symptoms noted 70% Immunostains showed 46% cases had T-cell phenotype, 36% null 18% B-cell phenotype. protein found 51% cases. IPI evenly distributed between ALK+ ALK− groups, except younger (median age, 30 v 61 years; P < .002). cohort included (44%), (42%), (14%) phenotypes. All 10 cytogenetic molecular evidence t(2;5) ALK+. 5-year overall (OS) entire 65%. OS 79% 46%, respectively ( .0003). Analysis only T-cell/null (n = 57) 93% 37% .00001). Univariate analysis ≤60 .007), normal serum lactate dehydrogenase (LDH) .00001), good performance status (Eastern Cooperative Oncology Group [ECOG] <2) .03), ≤1 extranodal site disease .012), score ≤3 .00001) improved OS. Although correlated positivity, multivariate LDH .0005), .005) predicted independently conclude heterogeneous disorder. However, independent predictor serves as biologic marker specific entity within spectrum ALCL.