PMC3968897_01
Course of blood lymphocytes according to symptoms and therapy.. Arrows underneath the graph represent treatment periods. Single arrow heads represent treatments that were begun or stopped outside of the time period represented on the graph. ASS: Anti-synthetase syndrome; MMF: mycophenolate mofetil.
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In March 2010 white blood cell count showed profound lymphopenia (486/mm 3, normal range 1,500 to 4,000/mm 3, Figure 1). Blood cell count showed 750 lymphocytes/mm 3 ( Figure 1). Toxoplasma gondii and Mycobacterium tuberculosis), and serologies (HIV, Borrelia and syphilis), were negative, as well as direct staining and cultures for bacteria and fungi. Blood immunophenotyping showed 673 CD4 + T cells/mm 3 (normal range 500-1,500), 82 CD8 + T cells/mm 3 (normal range 250-950) and 37 CD19 + B cells/mm 3 (normal range 100-600) ( Figure 1). The level of anti-Jo1 antibodies previously detected (Nov 2009, 7.4 In parallel, blood immunophenotyping showed partial normalization ( Figure 1).
Brain MRI and histopathology.. 
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B: Brain MRI at the onset of the simultaneous PML-IRIS showing white matter hyperintensities of the right frontal and parietal region (A, axial T2-WI) with multiple foci of gadolinium enhancement (B, axial T1-WI).
C: Brain MRI nine months after the onset of PML-IRIS showing sequellar lesions (axial T2-WI).
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E: Histopathology showing severe perivascular infiltrates (D, H&E x 200) with a vasculitic component (E, H&E x 630, star: fibrin deposit, arrow: neutrophils and serohematic material, arrowhead: lymphocytes infiltrating vessel wall).
F: Immunohistochemistry analysis (x400) showing perivascular inflammatory infiltrates positive for CD3 antibody (clone F7.2.38, DAKO).
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Brain axial T2-WI MRI revealed confluent subcortical white matter hyperintensities of the right frontal and parietal region ( Figure 2A). Axial T1-WI MRI displayed hypointensities with multiple foci of gadolinium enhancement ( Figure 2B). Neuropathological examination showed demyelinated lesions with axonal loss and a severe inflammatory reaction with a vasculitic component and endothelial damage ( Figure 2D-E). Perivascular and parenchymal inflammatory infiltrates showed a pronounced CD3 + T cell infiltrate ( Figure 2F) composed mostly by CD4 + T cells, in association with a few CD68 + macrophages/microglial cells and CD138 + plasma cells. The patient was fully independent without any neurological abnormalities, while MRI showed sequellar lesions ( Figure 2C).