Extranodal NK/T-Cell Lymphoma, Nasal Type—Case Report of 2 Cases

Abstract

A 17-year-old male was admitted to hospital for fever, right periorbital edema, purulent rhinorrhea, and nasal obstruction for 3 days ( Figure 1A). He had been under treatment with antibiotic and corticoid for rhinosinusitis, with no improvement. Previous surgery was performed 6 months before for ''nasal polyposis.'' The anatomopathological (AP) highlighted polypoid inflammatory mucosa.Upon examination, he presented with peripalpebral edema on the right, conjunctival hyperemia with preserved ocular mobility and visual acuity and painless subcutaneous nodules on the right hemiface. Nasoendoscopy showed no changes in the left or nasopharynx; in the right, edema and mucosa hyperemia obstructing the upper airway and purulent rhinorrhea.Contrast computerized tomography (CT) of sinuses and orbit revealed extraconal infiltrating and expanding injury compromising orbit and right periorbit, with eyeball proptosis; maxillary, sphenoid sinus, ethmoidal cells, frontal sinus, and nasolacrimal duct were obliterated; and infiltration of the skin and subcutaneous on the right was identified ( Figure 1B and C).Abdomen CT revealed splenomegaly (15.5 cm). Pelvis and chest CT were normal. Serologies, rheumatic tests, hemoculture, uroculture, and Mantoux were negative. During admission, cefepime was commenced for febrile neutropenia. Biopsy of the lesion via nasal endoscopy in the right nasal cavity was performed with Epstein-Barr virus (EBV) checking positive. There was not enough material for diagnosis through immunohistochemistry due to the large amount of necrotic material.There was a gradual worsening of pancytopenia, increase in lactate dehydronagenase (LDH), and episode of right severe epistaxis, which was controlled with nasal packing. Bone marrow biopsy was carried out due to the suspicion of lymphoma, with negative result. Prednisone was initiated in lower doses, and new biopsy of the lesion in the right nasal cavity was performed through lateral rhinotomy, anatomopathology examination identified lymphoma. Extranodal natural killer /T-cell lymphoma, nasal type (ENKL) was confirmed by immunohistochemistry. Treatment with radiotherapy and outpatient chemotherapy was commenced.After 2 months, the patient was admitted to hospital with fever and pancytopenia. A treatment with piperacillin and tazobactam was commenced. As no response was observed, antibiotic treatment was changed to cefepime. Fever persisted, general condition worsening progressed, liver and renal failure occurred. Nephrotoxic and hepatotoxic drugs were suspended, with no improvement in the condition. A new abdominal CT was requested for investigation of the clinical worsening, which suggested lymphoma progression with renal and hepatic infiltration. There was no response to new pushes of corticosteroid therapy. Patient died on the 17th day of admission. Case 2An 86-year-old female, healthy, was referred to otorhinolaryngologist after 3 months of admission at another hospital due to acute rhinosinusitis with no response to clinical treatment. She reported nasal o...

Two young patients with extranodal natural killer/T-cell non-Hodgkin lymphoma, nasal-type (ENKTL-NT) masquerading inflammatory processes: A case series

“…[ 1 3 ] However, an unusual presentation of ENKTL-NT cases was reported, and it is crucial to be rapidly recognized, considering its highly aggressive nature. [ 6 9 10 15 ] Delayed diagnosis may occur because of the clinical presentations of ENKTL-NT masquerading a variety of morphologies, as found in our patients. Besides, it has also formerly stated that patients with prominent ocular symptoms are more likely presented at the later stage, with periorbital swelling as the most common presentation.…”