A Case of Kikuchi's disease accompanied by Hemophagocytic Lymphohistiocytosis

ABSTRACT

Kikuchi's disease usually occurs in young women and is characterized by localized lymphadenitis (mostly cervical) usually associated with fever. It is considered a self-limited disease and most patients recover spontaneously within a few weeks to 6 months without any serious sequelae. However, patients with Kikuchi's disease require a systemic survey and regular follow-up for several years because it may be associated with other diseases such as systemic lupus erythematosus. To our knowledge, there are very few reports of Kikuchi's disease accompanied by hemophagocytic lymphohistiocytosis. Biopsy of a right cervical lymph node in a 35-year-old female who presented with fever and masses in the right cervical region showed necrotizing lymphadenitis and a diagnosis of Kikuchi's disease was reached. She was started on methylprednisolone pulse therapy (500 mg for 3 days) but developed generalized rash and fever. Laboratory data showed pancytopenia, elevation of serum transaminase and ferritin levels. Bone marrow and liver biopsy showed proliferation of histiocytes and Kupffer's cells engulfing lymphocytes, platelets and red blood cells, respectively. We report a case of Kikuchi's disease accompanied by hemophagocytic lymphohistiocytosis.