Case
An11-year old girl presented with vision deterioration and was diagnosed with uveitis. Chest computed tomography showed infiltration in her lung field, for which she was referred to our hospital. She also had fever(38.5°C). After the patient’s fever persisted, laboratory findings fulfilled criteria for hemophagocytotic syndrome (HPS) [1]. Positron emission tomography-magnetic resolution image (PET-MRI) scanning demonstrated several abnormal accumulations (Figure 1). Pathological investigation from sinusoid, lungs and kidney biopsies showed atypical lymphoid cells with positive for CD3,4,7,8,56, and negative for CD19,20 with positive for EBV-encoded RNA in situ hybridization. Final diagnosis was extranodal natural killer /T cell lymphoma, nasal type (ENKTL).
The patient was treated with dexamethasone, methotrexate, ifosfamide, L-asparaginase, and etoposide (SMILE) for six courses following umbilical cord blood stem cell transplantation (UCB-SCT) and has been in complete remission for 3 years.
ENKTL is rare in children. Previous reports have documented involvement of multiple organs, including the nasal cavity, skin, gastrointestinal tract, liver and lungs, but only a few cases have reported involvement of the eyes and kidneys. The most distinctive symptom was deterioration of sight. In terms of treatment, advanced ENKTL with HPS has been reported to have the worst prognosis. A strong regime with SMILE in addition to SCT can possibly improve [2], and proved successful in this patient.