Kikuchi-Fujimoto disease 4.5/5 (2)

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Kikuchi's

Kikutchi-Fujimoto disease with swollen lymph node on the neck. Khadanga S, 2014. Openi, CC BY NC SA 3.0

KIKUCHI-FUJIMOTO disease (histocytic necrotizing lymphadenitis)

Definition

Kikutchi-Fujimoto Disease (KFS) is a rare, benign, necrotizing lymphadenopathy (lymph node disease), most often on the neck of young women. Histology (tissue examination in biopsy) separates from cancer: histiocytic necrotizing. Unknown cause. The disease is in some cases associated with Systemic lupus erythematosus (SLE).

Occurrence

Most are young women, the disease can also affect men. Typically under 40 years of age at the onset, but reported between 6 - 80 years of age. The average age at onset is 30 years.

Symptoms

  • Swollen lymph nodes on the neck (100%, 1 - 2 cm in diameter, up to 7 cm described)
  • Fever (30-50%, 1-4 weeks) at debut
  • Night sweats
  • Dizziness
  • Headache
  • Vomit
  • Diarrhea
  • Weight Loss (10%)
  • Rash
  • Arthritis (5-7%)
  • Large liver and spleen (hepatosplenomegaly) (3%)
  • Bone marrow: Increased number of macrophages, no atypical cells
  • SLE Association occurs, but ANA test is usually normal

Medical examination

Blood tests

  • Many have normal tests. Low white blood cell count (Leukopenia, in 20-32%, Blood Reduction Response (SR) over 60mm in 40 - 70%, anemia in 23%)

Tissue test (biopsy) is crucial for the diagnosis

Diagnosis

Lymph node biopsy (distinguish from lymphoma: Hodgkin, non-Hodgkin).

  • Thin needle biopsy can be used. immunohistochemistry
    • CD68 + T lymphocytes are detected
    • Absence of neutrophils in the "necrotizing phase" differs from SLE

CT examination of swollen lymph nodes shows perinodular infiltration (81%), homogeneous contrast recording (83%)

Incorrect diagnosis? (Similar diseases / differential diagnoses)

Treatment / follow-up

No specific therapy. For severe symptoms can corticosteroids (Prednisone) be useful.

Check to exclude SLE development after a few years.

Prognosis

Self-limiting illness. Spontaneous decline after 1 - 4 months.

Literature


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