Relapsed EBV-negative aggressive NK cell Lymphoma of the orbit and scalp - A Case Report with review of literature

Abstract

Nasal NK/T-cell lymphoma is a rare extranodal large cell lymphoma that involves the nasal cavity. In majority of cases, Epstein Barr virus is present and Immunophenotype shows CD3 and CD56 positivity. Loco-regional relapse is very common with poor prognosis. We report a case of Relapsed NK cell Lymphoma of the orbit and scalp region in a young boy with EBV negativity, CD3 negativity and clinically aggressive disease. Despite aggressive treatment, our case relapsed early and was complicated by prolonged pancytopenia, CNS involvement and patient died less than one year following diagnosis. NK cell lymphoma is an aggressive cancer with high mortality. Multidisciplinary modalities are essential to treat the NK cell lymphoma with radiotherapy, chemotherapy, newer agents, and hematopoietic stem cell transplantation.