NK-Cell Enteropathy ICD-10: K63.8

Natural killer (NK) cell enteropathy is a benign NK cell lymphoproliferative disease and characterized by milder abdominal symptoms (abdominal pain, diverticulosis, constipation, reflux).

Etio: Causative lesions in the mucosal layer of the gastrointestinal tract derive from NK cells and are often misinterpreted as NK or T-cell lymphoma .

Xiao W et al (2019) detected recurrent JAK3 K563_C565del mutations in 30% of cases. Furthermore, several studies demonstrated that mutations in the JAK-STAT pathway occur in up to 30% of NK or T-cell lymphomas. The identification of the same mutation in a variety of diseases ranging from indolent to aggressive suggests that additional molecular mechanisms contribute to these diseases.

1. Gasparini VR et al (2020) A high definition picture of somatic mutations in chronic lymphoproliferative disorder of natural killer cells. Blood Cancer J 10:42.
2. Xiao W et al. (2019) Recurrent somatic JAK3 mutations in NK-cell enteropathy. Blood 134:986-991.
3. Xia D et al. (2019) NK-cell enteropathy and Similar Indolent Lymphoproliferative Disorders: A Case Series With Literature Review. Am J Clin Pathol 151:75-85.