Orbiting a Diagnosis

Given the constellation of MRI findings in the context of the patient's history of CLL/SLL and CBC demonstrating progressive lymphocytosis from 18.2 K/uL to 39.9 K/uL over a 3-month period, there was suspicion for an infiltrative etiology of the patient's optic perineuritis and orbital disease (1). However, in light of the patient's atypical pain and orbital symptomatology, a broad infectious and inflammatory laboratory panel was obtained, along with an orbital fat biopsy to assess for infection and/or leukemic infiltration and a concurrent temporal artery biopsy to rule-out giant cell arteritis. (Figures 4-7) Assays for syphilis, tuberculosis, epstein-barr virus, cytomegalovirus, lyme disease, aspergillus, herpes simplex, and toxoplasma were negative. No organisms were seen on orbital pathology, and temporal artery biopsy was negative for GCA. The patient's visual acuity remained at 20/40 with 3-4+ disc edema OS on hospital day 4, but interval worsening of visual acuity to 20/30 with 1+ disc edema was noted OD. In the setting of unrevealing serologies and pathology, IV methylprednisolone 1g daily was initiated, without meaningful improvement in visual acuity over the following 3 days. A lumbar puncture subsequently revealed abundant cryptococcus and lambda restricted B cells (Figures 8-9). Corticosteroid was discontinued, and IV amphotericin B and flucytosine were initiated. The patient's visual acuity improved to 20/20 OU the following day. His hospital course was complicated by presumed Immune Reconstitution Inflammatory Syndrome after initiation of antifungal therapy, with symptoms of diffuse rash, altered mental status, and hemodynamic instability. One month following discharge on oral flucytosine therapy, the patient's visual acuities were 20/20 OD and 20/30 OS with no rAPD, full colors, full and painless ocular motility and normal appearing optic discs. These data support a final diagnosis of infiltrative perineuritis secondary to cryptococcal meningitis with aggregation of lymphoma cells at the area of inflammation.