Extranodal NK/T-Cell Lymphoma with Cutaneous Presentation and Fever of Unknown Origin

Extranodal NK/T-cell lymphoma is a rare subtype of extranodal large cell lymphoma that typically involves the nasal cavity, nasopharynx, and the upper aerodigestive tract where as non-nasal NK-cell lymphoma involves any other locations of the body. Extranodal NK/T-cell lymphoma is exceeding rare in Western Countries, with most documented cases occurring in Asia. The disease often involves the skin, gastrointestinal tract, and testis. Involvement of lymph nodes, blood, and bone marrow can occur and typically overlaps with Aggressive NK-cell leukemia. Diagnosis is typically made with biopsy and immunostaining, however given the extensive necrosis seen in these tumors this often requires multiple biopsies. Typically Epstein Barr virus is present and immunophenotype shows CD56. Unfortunately this newly recognized distinctive lymphoma has not yet had a defined optimal therapy, however SMILE or CHOP and generally recommended. Prognosis is generally very poor, with the Korean Prognostic Index as the most widely used prognostic tool. Here we present a case of a 74-year-old female who presented with erythematous scalp raised patches and persistent fevers, chills, weight loss and night sweats found to have Extranodal NK/T-cell lymphoma. Bowers J, Dorsey JT, Wolanin S, Lamar Z and Ruiz J* Department of Medicine/Oncology, Wake Forest School of Medicine Health Sciences, USA Figure 1: Pre Chemotherapy. Ruiz J, et al. Clinics in Oncology Chemotherapy and Radiotherapy Remedy Publications LLC., | http://clinicsinoncology.com/ 2016 | Volume 1 | Article 1095 2 or immunophenotypically aberrant T cell population were identified. She had a previous non-diagnositic FNA of the right temporal nodule re-examined at our facility which showed rare atypical cells of lymphoid origin. A bone marrow biopsy was performed which showed hypercellular marrow without evidence of malignancy. A punch biopsy of the left frontal scalp was performed by dermatology and showed suppurative neutrophilic dermatitis with PAS, GMS, and AFB stains negative for organisms. At this time the patient was administered intravenous prednisone with improvement of the skin lesions. Given the clinical suspicion of underlying malignancy the patient underwent a wider excisional biopsy of her left scalp prior to treatment (Figures 1-3). The infiltrate was composed of large and atypical T-cells which expressed CD3, CD8, granzyme B and weakly CD56. The malignant cells were negative for BetaF1 and TCRgamma/delta (Figure 4). The malignant cells were positive for EBV by Epstein-Barr virus-encoded RNA in situ hybridization test and PCR for T-cell receptor gamma gene rearrangement was polyclonal. The findings were most consistent for EBV-associated extranodal NK/T cell lymphoma (Figure 5). Following the diagnosis, a staging PET scan revealed widespread metastatic disease with multiple foci of uptake in soft tissues, muscles, and lymph nodes. There was no nasal involvement. Patient was categorized as Risk Group 4 (elevation in LDH, stage >III, and B symptoms), which carries a 6.6% 5 year overall survival rate 6. The patient was treated with EPOCH (etoposide 89 mg, doxorubicin 17.8 mg, vincristine 0.5 mg, cyclophosphamide 750 mg/ m2, and prednisone 60 mg/m2) with immediate clinical response of her skin lesions.