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.