Diagnostic and Therapeutic Challenges

Abstract

A 35-year-old Vietnamese physician presented to the clinic in December 2010 with reduced vision in both the eyes for 4 months. His medical history is significant for a myelodysplastic syndrome, refractory anemia with excess blasts-2, which transformed into acute myeloid leukemia, for which he received 3 cycles of azacitidine and 1 cycle of FLAG chemotherapy (fludarabine, cytarabine, and granulocyte colony-stimulating factor regimen) before eventually undergoing a reduced intensity-matched unrelated donor stem cell transplant in June 2010. Before his transplant, the patient was found to be positive for cytomegalovirus (CMV) and herpes simplex virus in the serum; the donor was also positive for CMV. After transplantation, he remained on cyclosporine (75/50 mg 2 times a day) for immunosuppression. His peritransplant course was complicated by Aspergillus pneumonia in April 2010, systemic CMV activation in July 2010 for which he was started on oral valganciclovir, and line-related (Hickman) Klebsiella sepsis in September 2010.The patient began to experience a painless reduced vision in the left eye starting in late August 2010. On evaluation, his visual acuity in the left eye was 20/160. Retinal examination of the left eye revealed increased attenuation of the vessels with flecken pigmentary changes, as well as intraretinal hemorrhages. Fluorescein angiogram showed attenuation of the arteries and veins in the left eye with capillary hyperfluorescence with no significant leakage noted in the late phase (Figure 1, August 2010). As he was positive for CMV in the serum, CMV vasculitis was suspected, and he was started on intravenous ganciclovir. Anterior chamber tap was negative for CMV, herpes simplex virus, and varicella zoster virus.Two weeks later, in September 2010, he was admitted for Klebsiella sepsis. Immediately following recovery, the visual acuity in the left eye worsened to counting fingers; the visual acuity in the right eye was 20/32, although an inferior field defect was detected. These changes were attributed to a central retinal artery occlusion in the left eye and branch retinal artery occlusion in the right eye (presumed to have happened when he was admitted to the intensive care unit for sepsis). He was evaluated for possible infectious, hypercoagulable and embolic conditions to ascertain the possible cause of the vaso-occlusive pathology.Chest computerized tomography and blood and urine cultures were negative for signs of infection. Magnetic resonance angiography showed 60% stenosis of the left external carotid and 32% stenosis of the right external carotid; however, ultrasound doppler of the carotids did not reveal any pathology. Serial electrocardiograms and holter monitoring were normal. Transthoracic echocardiogram showed no evidence of a patent foramen ovale. Hypercoagulable workup showed positive anticardiolipin antibodies and lupus anticoagulant. C-reactive protein and erythrocyte sedimentation rate were within normal limits with low protein S and normal protein C, along with other normal serol...