Risk stratification of adult T-cell leukemia/lymphoma using immunophenotyping

Abstract

Adult T-cell leukemia/lymphoma (ATL), a human T-lymphotropic virus type 1 (HTLV-1)-associated disease, has a highly variable clinical course and four subtypes with therapeutic and prognostic implications. However, there are overlapping features between ATL subtypes and between ATL and nonmalignant (non-ATL) HTLV-1 infection complicating diagnosis and prognostication. To further refine the diagnosis and prognosis of ATL, we characterized the immunophenotype of HTLV-1-infected cells in ATL and non-ATL. A retrospective study of peripheral blood samples from 10 HTLV-1-uninfected subjects (UI), 54 HTLV-1-infected patients with non-ATL, and 22 with ATL was performed using flow cytometry. All patients with ATL had CD4⁺ CCR4⁺ CD26⁻ immunophenotype and the frequency of CD4⁺ CCR4⁺ CD26⁻ T cells correlated highly significantly with the proviral load in non-ATL suggesting CD4⁺ CCR4⁺ CD26⁻ as a marker of HTLV-1-infected cells. Further immunophenotyping of CD4⁺ CCR4⁺  CD26⁻ cells revealed that 95% patients with ATL had a CD7⁻ (≤30% CD7⁺ cells), whereas 95% HTLV+ non-ATL had CD7⁺ (>30% CD7⁺ cells) immunophenotype. All patients with aggressive ATL had a CCR7⁺ (≥30%), whereas 92% with indolent ATL and 100% non-ATL had a CCR7⁻ (<30%) immunophenotype. Patients with nonprogressing indolent ATL were CD127⁺ but those with progressive lymphocytosis requiring systemic therapy had a CD127⁻ (≤30%) immunophenotype. In summary, HTLV-1-infected cells have a CD4⁺ CCR4⁺ CD26⁻ immunophenotype. Within this population, CD7⁻ phenotype suggests a diagnosis of ATL, CCR7⁺ phenotype identifies aggressive ATL, while CCR7⁻ CD127⁻ phenotype identifies progressive indolent ATL.

Adult T-cell leukemia/lymphoma (ATL) has a highly variable clinical course and better prognostic markers are urgently required. Our study has shown human T-lymphotropic virus type 1 (HTLV-1)-infected cells have a CD4⁺ CCR4⁺ CD26⁻ immunophenotype. Within this population, CD7⁻ immunophenotype suggests a diagnosis of ATL, CCR7⁺ immunophenotype identifies aggressive ATL, while CCR7⁻ CD127⁻ immunophenotype identifies progressive indolent ATL.

from #AlexandrosSfakianakis via Alexandros G.Sfakianakis on Inoreader http://ift.tt/2hyaAyq
via IFTTT