Increased Intracranial Pressure As The Presentation Of Evans Syndrome In
A 4-Year-Old Boy
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