Abstract
A 20 year-old female was admitted to our clinic with a one month history of fever elevation up to 40°C. Laboratory findings on admission were as follows: Hb 8.7g/dl, platelet count 130,000/μl, WBC 3,000/μl with 26% abnormal lymphoid cells. These lymphoid cells had abundant pale cytoplasm with large azurophilic granules and a large nucleus. Flow cytometric analysis of lymphocytes expressed OKT-11, OKIa1, and N901 (NKH-1) which is considered to detect a portion of the NK cell subset.
Chemotherapy regimen with VEPA was almost ineffective. The patient died of perforating esophageal ulceration, interstitial pneumonia, and intestinal bleeding 4 months after admission in spite of intensive supportive therapy in ICU station.
We considered that such a unique finding associated with abnormal immune reaction and poor prognosis might belong to a new clinical entity which originate from NK cell subset.
