Extranodal Natural Killer/T-Cell Lymphoma of the Small Intestine Associated With Reactive Hemophagocytic Syndrome: Case Report and Literature Review

Primary extranodal natural killer (NK)/T-cell lymphoma (NTCL) of the gastrointestinal tract is extremely rare. It has an aggressive clinical course with a high mortality rate; early diagnosis is usually difficult because patients show nonspecific symptoms. We describe a case of primary NTCL of the small intestine in a 45-year-old ethnic Korean woman who has clinical features similar to those of inflammatory bowel disease (IBD). Laparotomy revealed a perforated jejunum and numerous masses in the mesentery. NTCL was diagnosed by a pathologist based on the results of immunohistochemical staining and the detection of Epstein-Barr virus encoded messenger RNA (mRNA) via in situ hybridization. On examination, a bone marrow sample showed diffuse histiocytic proliferation with hemophagocytosis. Although the patient received dose-intense chemotherapy and supportive treatment, she died 21 days after her diagnosis. Herein, we report a rare case of primary NTCL of the small intestine with reactive hemophagocytic syndrome and review the literature on this rare disease.