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Increased Intracranial Pressure As The Presentation Of Evans Syndrome In A 4-Year-Old Boy
  • Lauren Herschbein,
  • Hillary Raynes,
  • Kayleen Bailey
Lauren Herschbein
Mount Sinai School of Medicine

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Hillary Raynes
Icahn School of Medicine at Mount Sinai
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Kayleen Bailey
Mount Sinai School of Medicine
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Evans syndrome (ES) is a combination of autoimmune cytopenias. Seldom patients with ES have been described as having neurological manifestations. We describe a case of ES presenting as increased intracranial pressure in a 4-year-old boy. This patient failed his routine vision screening and was found to have bilateral papilledema. Labwork: neutropenia, thrombocytopenia, and anemia with warm autoantibody IgG (Direct Coombs IgG 2+, C3 negative, weak para-agglutination). Brain MRI/MRA/MRV: increased intracranial pressure. CT abdomen/pelvis: enlarged paraaortic lymph nodes, without splenomegaly. Bone marrow biopsy/aspirate and LN biopsy: negative for evidence of malignancy or infection. Patient was managed with prednisone and topiramate.