The DP-T-cell phenotype occurs in 21% of patients with T-PLL 3. T-PLL is a highly aggressive heterogeneous disease with short life expectancy, even in patients who initially present an indolent course. Transient or persistent increases in DP-T-cell numbers have been recognised in the PB of patients with viral infections, collagen diseases, neoplasic conditions and also in normal individuals, although little is known about the antigen specificity, major histocompatibility complex restriction and function 2. The DP-T phenotype is characteristic of thymic cortical T-cells, which are selected positively and negatively to generate two major lineages of mature αβ T-cells, defined by the mutually exclusive expression of CD4 or CD8 coreceptors. This pathological pattern is not specific to any particular disorder, but reflects an inflammatory process resulting from lung disease 1.ĬD4+CD8+ DP-T-cells are usually present in very low numbers (2–6%) in peripheral blood (PB), but increased frequencies have been reported in apparently healthy individuals and in various clinical conditions. The lesions occur predominantly within the alveolar spaces and bronchiolar lumen. These cells have similar features to indolent T-prolymphocytic leukaemia (T-PLL) cells.īOOP is pathologically characterised by buds of granulation tissue in the distal air spaces that progress from fibrin exudates to loose collagen-containing fibroblasts. The present study describes an unusual case of recurrent lung infiltration showing bronchiolitis obliterans and organising pneumonia (BOOP) with atypical double-positive (DP)-T-lymphocyte lung infiltration that was also present in peripheral blood.
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