KIKUCHI FUJIMOTO DISEASE

DEFINITION

Benign disease  characterized by lymphadenopathy with distinctive  histological features.

SYNONYMS :

INCIDENCE   Worldwide distribution, however high incidence in Asians.

EPIDEMIOLOGY -  Most patients are  young women , the average age at presentation 27years.

ETIOLOGY – unknown.

CLINICAL FEATURES -  Unilateral tender post cervical lymphadenopathy, fever, rash, anemia, and leukopenia. rarely presents with the atypical lymphocytosis.

MORPHOLOGY:

• Patchy Lymph node involvement , usually centered  in paracortical areas
• Atleast occasional crescentic histiocytes
• Fibrinoid necrosis surrounded by immunoblasts , histiocytes and plasmacytoid dendritic cells.
• Extracellular apoptotic/karyorrhectic debri
• Absence of eosinophils,polymorphonuclear cells and plasma cells

EXTENSIVE LYMPH NODE INVOLVEMENT WITHOUT EFFACEMENT OF LYMPH NODE ARCHITECTURE AND RESIDUAL FOLLICLES (SCANNER VIEW).

PROLIFERATIVE TYPE WITHOUT NECROSIS (LOW POWER)

NECROTIZING TYPE WITH ABUNDANT NECROTIC FOCI ( LOW POWER VIEW )

HIGH POWER VIEW SHOWING ABUNDANT KARYORRHECTIC DEBRI WITH ABSENCE OF NEUTROPHILS

HIGH POWER VIEW SHOWING CRESCENTIC HISTIOCYTES

HISTOLOGIC SUBTYPES :

IMMUNOPHENOTYPE

plasmacytoid dendritic cells express CD 2 , CD4 , CD 43 , CD68  and CD123.

DIAGNOSIS

LYMPH NODE BIOPSY

DIFFERNTIAL DIAGNOSIS

PROGNOSIS

Some  patients develop recurrent lymphadenopathy

Rare patients, usually with an underlying immunodeficiency , have a severe illness with a poor outcome.

MANAGEMENT- nearly all patients have a self limited illness requiring no specific therapy. 

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