Successful treatment of nasal-type extra-nodal natural killer/T cell lymphoma with simultaneous involvement of the thyroid, liver, and pancreas

Abstract

Dear Editor, Extra-nodal NK/T cell lymphomas (ENKLs) reflect their putative cellular origins from both NK cells and T cells [1]. For stage III/IV lymphomas, chemotherapy is the mainstay of treatment. However, conventional anthracycline-based regimens are ineffective. Recommended chemotherapy protocols are based on the use of L-asparaginase combined with other effective drugs [2]. Herein, we report a case of nasal-type ENKL with thyroid, liver, and pancreas involvement, which has never previously been reported. The treatment was Lasparaginase with an anthracycline-based regimen that led to a favorable response. A 54-year-old man presented with abdominal fullness for 2 months accompanied with poor appetite, intermittent steatorrhea, and weight loss of 9 kg. The initial laboratory data disclosed hyperbilirubinemia with abnormal liver biochemistry profiles (Table 1). No evidence of viral hepatitis was found, and the autoimmune profiles were all within normal ranges. Computed tomography (CT) revealed a diffuse puffy appearance of the pancreatic parenchyma (Fig. 1a). Fludeoxyglucose positron emission tomography (FDG-PET) revealed increased uptake in the pancreas, liver, thyroid glands, and right nasal cavity (Fig. 1b). Subsequent endoscopic ul t rasonography examinat ion and endoscopic ultrasonography–guided fine-needle aspiration biopsy for the pancreatitis work-ups, however, did not produce a conclusive result. The patient was subsequently treated with prednisolone empirically for autoimmune pancreatitis. The response was poor, as demonstrated by the deterioration of the laboratory profiles within 10 days (Table 1). Liver biopsy was therefore performed. The pathology tests revealed atypical lymphocyte infiltration with strong expression of both CD3 and Epstein– Barr virus (EBV)-encoded RNAwith negative CD56 expression. A final diagnosis of nasal-type extra-nodal NK/T cell lymphoma (ENKL) was thus made. Induction chemotherapy with two cycles of L-asparginase, cyclophosphamide, doxorubicin, vincristine, and prednisolone (L-CHOP) treatment resulted in favorable clinical response with the regression of all clinical symptoms, abnormal laboratory profiles, and EBV viral loads. Two cycles of L-asparaginase, etoposide, dexamethasone, cytarabine, and cisplatin treatment (LESHAP) were then administered for consolidation. After the chemotherapy treatments, the total bilirubin level returned to normal and no EBV viral load was detected (Table 1). Repeated CT confirmed regression of the prior pancreas findings (Fig. 1c), and FDG-PET revealed that the abnormalities observed in the pancreas, liver, thyroid glands, and nasal cavity were resolved (Fig. 1d). The patient subsequently received upfront autologous peripheral blood stem cell transplantation (auto-PBSCT) 5 months after the diagnosis was made. The patient remains in complete remission 14 months after the auto-PBSCT. Extra-nasal-type ENKL accounts for the remaining 20% of patients and involves the intestines, skin, testes, lungs, eyes, adrenal glands, brain, breasts, tongue, thyroid, and pancreas [3–8]. Our case exhibited simultaneous involvement of the thyroid, pancreas, and liver, which has never been reported before. The options for frontline therapy were limited because * Shang-Ju Wu [email protected]