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Extranodal NK/Tcell lymphomas (ENKTL) are rare, aggressive lymphomas. The most common primary site of involvement is the nasal cavity, nasopharynx and paranasal sinuses. The other sites of primary involvement are skin, gastrointestinal tract and testis. Advanced disease can show lymph node, bone marrow and peripheral blood involvement. We report a case of ENKTL of the jejunum, showing peripheral pancytopenia and haemophagocytosis in the bone marrow. The intestine showed multiple intestinal perforations, with evidence of infiltration by lymphoma with coexistent strongyloides infestation. The patient showed disseminated disease in the form of lymphadenopathy and had a rapidly downhill course and expired on 5th day of admission. We also discuss the problems encountered by the pathologist in diagnosing these uncommon lymphomas.

Extranodal NK/T Cell Lymphoma of the Jejunum Complicated by Hemophagocytic Syndrome: Practical Problems Encountered by a Pathologist