Leukaemia presenting as branch retinal artery occlusion & op
The present case has been reported in BMJ.

A previously healthy 52-year-old man presented to the emergency department with acute painless vision loss in the inferior visual field of his left eye. Review of systems was positive for daily headaches over the past 3 months but otherwise negative for neurological symptoms. Bedside examination was notable for a visual acuity of 20/40 in addition to a relative afferent pupillary defect in the left eye.

Initial fundus examination revealed optic disc oedema, which appeared infiltrative, in addition to superior retinal whitening. Subsequent fluorescein angiography showed a superior branch retinal artery occlusion (BRAO). Optical coherence tomography angiography confirmed superior retinal capillary non-perfusion with arteriolar narrowing at the optic disc border.

Initial laboratory workup in the emergency department revealed a white cell count of 62.1K with 82.5% lymphocytes. Initial cerebrospinal fluid (CSF) analysis on lumbar puncture contained 70 mg/dL glucose, 39 mg/dL protein and 17 white blood cells per μL with 88% lymphocytes. A lymph node biopsy showed proliferation of small lymphocytes positive for B cell markers consistent with chronic lymphocytic leukaemia (CLL) and subsequent CSF flow cytometry revealed 84% CD19 +B cells confirming central nervous system (CNS) involvement.

The patient received four doses of intrathecal chemotherapy with methotrexate/cytarabine/hydrocortisone in addition to one cycle of fludarabine and rituximab systemically. He began a course of intravenous steroids for 3 days for his vision loss with transition to oral prednisone thereafter. Three weeks after initial diagnosis, his visual acuity had improved to 20/30 with resolution of his optic disc oedema although his inferior visual field deficit remained.

The patient was continued on intrathecal chemotherapy as an outpatient in addition to one dose of ibrutinib. Three months after initial diagnosis, the patient was sent to the emergency department for progressive shortness of breath and night sweats. He was admitted for broad-spectrum antibiotics and was subsequently found to have Aspergillus pneumonia. The patient ultimately developed acquired respiratory distress syndrome, acute renal failure and cerebral oedema and succumbed to his illness during this admission.

Learning points
• Optic disc oedema and infiltration in chronic lymphocytic leukaemia (CLL) are rare but can occasionally be the presenting sign of this disorder.

• Systemic workup in cases of atypical optic disc oedema and branch retinal artery occlusion can aid in the timely diagnosis of aggressive systemic disease.

• This is the first case report of simultaneous optic disc oedema and branch retinal artery occlusion as the initial presentation of CLL.


Read more here: http://casereports.bmj.com/content/2018/bcr-2018-227691.full
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