Korean J Hematol.  2009 Dec;44(4):325-329. 10.5045/kjh.2009.44.4.325.

A Case of Kikuchi Fujimoto's Disease Accompanied by Hemophagocytic Lymphohistiocytosis

Affiliations
  • 1Department of Internal Medicine, Hanyang University College of Medicine, Seoul, Korea. drbbpark@gmail.com

Abstract

Kikuchi's disease and hemophagocytic lymphohistiocytosis (HLH) present different clinical characteristics, especially in prognosis, although both diseases have the clinical similarity in initial presentations. Kikuchi's disease usually has a self-limiting clinical course, but HLH can be fatal. Accordingly, it is important that the differential diagnoses and decision as to initial treatment be made as soon as possible, at the time of clinical presentation. In the case of Kikuchi's disease accompanied with HLH, the decision concerning initial treatment can be very difficult, because these cases have been rarely reported and the prognosis is unpredictable. We report a case of a 21-year-old female diagnosed with Kikuchi's disease accompanied with HLH. Treatment involved steroid therapy, as for treatment of HLH. Recovery was complete. Kikuchi's disease with HLH can be completely treated with more aggressive therapy than used for Kikuchi's disease alone.

Keyword

Kikuchi's disease; Hemophagocytic lymphohistiocytosis; Hemophagocytic syndrome

MeSH Terms

Diagnosis, Differential
Female
Histiocytic Necrotizing Lymphadenitis
Humans
Lymphohistiocytosis, Hemophagocytic
Prognosis
Young Adult

Figure

  • Fig. 1. (A,B) BM aspiration: the monohistiocytes are increased in number, and some of them phagocytize RBC and platelet. (×400/×1,000, Wright-Giemsa stain) (C) BM section: the BM section is hypocellular (<10%) (×100, H&E stain) (D) BM section: EBV-LMP immunohistochemical staining result as positive in BM (×100, BM immunohistochemical stain).

  • Fig. 2. Cerviacl lymph node biopsy: (A) Lymph node shows focal necrosis (×40, H&E stain), (B) karyorrhectic debris, cre-sentic histiocytes, small lymphocytes with absence of neutrophils and eosinophils (×100, H&E stain).


Cited by  1 articles

Hemophagocytic lymphohistiocytosis with recurrent Kikuchi-Fujimoto disease
Sang Min Lee, Young Tae Lim, Kyung Mi Jang, Mi Jin Gu, Jong Ho Lee, Jae Min Lee
Yeungnam Univ J Med. 2020;38(3):245-250.    doi: 10.12701/yujm.2020.00654.


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