Yeungnam Univ J Med.  2021 Jul;38(3):245-250. 10.12701/yujm.2020.00654.

Hemophagocytic lymphohistiocytosis with recurrent Kikuchi-Fujimoto disease

• Lee SM
• Lim YT
• Jang KM
• Gu MJ
• Lee JH
• Lee JM

• KMID: 2518668
• DOI: http://doi.org/10.12701/yujm.2020.00654

Abstract

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a self-limiting lymphadenitis. It is a benign disease mainly characterized by high fever, lymph node swelling, and leukopenia. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease with clinical symptoms similar to those of KFD, but it requires a significantly more aggressive treatment. A 19-year-old Korean male patient was hospitalized for fever and cervical lymphadenopathy. Variable-sized lymph node enlargements with slightly necrotic lesions were detected on computed tomography. Biopsy specimen from a cervical lymph node showed necrotizing lymphadenitis with HLH. Bone marrow aspiration showed hemophagocytic histiocytosis. The clinical symptoms and the results of the laboratory test and bone marrow aspiration met the diagnostic criteria for HLH. The patient was diagnosed with macrophage activation syndrome—HLH, a secondary HLH associated with KFD. He was treated with dexamethasone (10 mg/m2/day) without immunosuppressive therapy or etoposide-based chemotherapy. The fever disappeared within a day, and other symptoms such as lymphadenopathy, ascites, and pleural effusion improved. Dexamethasone was reduced from day 2 of hospitalization and was tapered over 8 weeks. The patient was discharged on day 6 with continuation of dexamethasone. The patient had no recurrence at the 18-month follow-up.

Keyword

Hemophagocytic lymphohistiocytosis; Kikuchi–Fujimoto disease; Necrotizing lymphadenitis

Figure

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  Fig. 1. (A) Neck computed tomography (CT) shows scattered, variable-sized lymph node enlargements (arrow) on both neck at levels Ⅰ to Ⅳ. (B) Chest CT shows a small amount of bilateral pleural effusion (arrows).

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  Fig. 2. Histological findings of the cervical lymph node. The biopsy specimen shows necrotizing lymphadenitis with karyorrhectic nuclear debris (arrows) (hematoxylin and eosin stain, ×400).

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  Fig. 3. (A) Peripheral blood smear shows atypical lymphocytes (arrow) (Wright’s stain, ×1,000). (B) Bone marrow aspirate smear shows hemophagocytic histiocytes (arrow) engulfing granulocytes and red blood cells (Wright’s stain, ×1,000).

Reference

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