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Lack of efficacy: case report An 8-year-old girl exhibited lack of efficacy during treatment with aciclovir and foscarnet for Epstein-Barr virus (EBV) and varicella meningoencephalitis. The girl had a history of developmental delay, unsteady gait and slurred speech and dysarthria. She also had a history of recurrent infections. Subsequently, she was diagnosed with systemic lupus erythematosus (SLE) and lupus nephritis. Therefore, she was treated with hydroxychloroquine, unspecified corticosteroid and mycophenolate mofetil with insignificant improvement. She also had underlying purine nucleoside phosphorylase (PNP) deficiency. Haematopoietic stem cell transplantation (HSCT) was scheduled. Before the stem cell transplantation, she developed refractory varicella zoster skin infections and Aspergillus pneumonia. Subsequently, she was hospitalised due to worsening of her clinical condition. Following investigations, she was diagnosed with varicella and EBV meningoencephalitis along with atypical malignant cells. She started receiving IV aciclovir and IV foscarnet [dosages not stated]. However, the meningoencephalitis was resistant to acyclovir and foscarnet , suggesting lack of drug effect. The onset of these all infections were attributed to PNP deficiency. A brain CT positron emission tomography (PET) scan revealed diffuse patchy signal changes involving the cingulate gyrus, genu of corpus callosum and crossing to the contralateral side. MRI showed the lesion, which had restricted diffusion in the diffusion-weighted imaging (DWI). Based on these findings, EBV-related CNS lymphoma was noted. Her condition further deteriorated. Subsequently, she died due to multiorgan failure and severe encephalopathy. Al-Saud B, et al. A Case with Purine Nucleoside Phosphorylase Deficiency Suffering from Late-Onset Systemic Lupus Erythematosus and Lymphoma. Journal of Clinical 803499564 Immunology 40: 833-839, No. 6, Aug 2020. Available from: URL: http://doi.org/10.1007/s10875-020-00800-y

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