Case Presentation:
A 23 year-old Guatemalan man presented to the hospital with right sided facial swelling, vesicular lesions on the eyelids and ulcers on the tongue. He was admitted with seven similar episodes during the past three months and was treated with steroids, antibiotics, antihistamines and also antiviral therapy for possible cellulitis and Herpes associated angioedema. His symptoms would partially resolve with steroids but would recur as soon as he completes the regimen. He has no significant PMH or FH, no H/O allergy to medications or food products. Vital signs were normal. On examination, there was swelling of right side of his face including the periorbital area, with crusted lesions on the eyelids, cheeks and shallow ulcers on the tongue. Urticaria was absent. Direct laryngoscopy revealed severe mucosal edema in nasal cavity, oropharynx, larynx and vocal cords. He was in moderate respiratory distress and was intubated for airway protection. CBC, CMP, C3, C4 and C1 esterase levels were all normal. HIV testing was negative. Excision biopsy of the enlarged cervical lymph node was performed. There were large atypical lymphoid cells that were positive for CD3, CD2, CD30, CD 56 and negative for CD4 and CD8. Epstein-Barr encoding region (EBER) in-situ hybridization stained clusters of atypical cells. The pathology and immunostaining were consistent with extranodal Natural Killer T-cell lymphoma (NKTCL) nasal type and it was confirmed by NIH pathology laboratory. Patient was started on SMILE regimen, which includes dexamethasone, methotrexate, ifosfamide, L-asparaginase, and etoposide.
Discussion:
NKTCL is an extremely uncommon disease, but usually shows a highly aggressive clinical course. The disease is much more frequent in Asian and Latin American countries than in Western countries, and is universally associated with Epstein–Barr virus infection. Tumor grows and invades subcutaneous tissue with lymphomatous infiltrate that follows the blood vessels and presents like angioedema. Compared with other subtypes of lymphoma found in the head and neck region, NKTCL carries a much higher mortality and responds less well to traditional chemotherapy and radiotherapy regimens.
Conclusions:
As hospitalists, it is not uncommon to encounter patients presenting with Angioedma. The median survival time for untreated NK/T-cell lymphoma is only one year and hence it is very important to recognize this rare condition in patients presenting with recurrent angioedema.