Fig. 1From: An unusual presentation of purine nucleoside phosphorylase deficiency mimicking systemic juvenile idiopathic arthritis complicated by macrophage activation syndromeImmunological functional tests of the patient. Intracellular expression of perforin in CD56+ NK cells was assessed by flow cytometry (a). Degranulation potential of CD56+ NK cells was assessed by flow cytometry after overnight co-culture with target cells K562. Plots show the surface expression of CD107a on unstimulated and stimulated NK cells (b). Lymphocytes subsets were analysed by flow cytometry: T cells were identified as CD3+ CD56− CD19−; within the CD3+ gate, γδ-T cells were gated as CD3+ γδ-TCR+ αβ-TCR− (c). T cell proliferation was assessed by a standard [3H]-thymidine incorporation assay following stimulation with anti-CD3 (OKT3) or PHA (d). The results are shown for first determination while on 2 mg/Kg of methylprednisolone. They were repeated on a lower dose of oral prednisone (0.25 mg/Kg) with similar resultsBack to article page