Benign, histiocytic necrotizing lymphadenitis usually
affecting young women. Unknown cause. Often mistaken for lymphoma or Hodgkin
disease. The disease resolves spontaneously within 2 to 3 months, and
relapse is uncommon.
Histiocytic necrotizing lymphadenitis.
It may account for 5 to 7% of all diagnoses among
pathologic specimens of abnormal lymph nodes. The majority of patients are
younger than 30 years but only 5 to 10% are younger than 21 years,
although patients from 19 months to 75 years of age have been reported.
Male-to-female ratio is 1:4. Most frequent in Japan where it was first
Unknown. Cytotoxic T cells expressing the
cytolytic protein perforin infiltrate the affected lymph node(s); apoptosis
induced by those T cells probably is the cause of the necrosis. The cause of
this hyperimmune or autoimmune reaction has not been identified, yet.
By histologic examination of the lymph node:
paracortical patchy zones of eosinophilic fibrinoid necrosis and abundant
karyorrhexis. Necrotic areas are surrounded by a mixed lymphohistiocytic
The most frequent presentation is cervical
lymphadenopathy, which often is tender or painful on palpation. It may
fluctuate in size. Hepatosplenomegaly may be present. Fever is predominant
in 30 to 50% of cases. Weight loss, night sweats, and a rash may occur.
Hematologic investigations show leukopenia, often with lymphocytosis, and
atypical lymphocytes in the peripheral blood; levels of C-reactive protein,
lactate dehydrogenase, and transaminases are raised. Spontaneous resolution
of signs and symptoms occurs within a few months; no specific therapy is
needed. Relapsing bouts of lymphadenopathy occur in 3 to 4% of cases. A
beneficial effect of systemic steroids has been reported. Rare cases of
associated myocarditis, aseptic meningitis, or ataxia have been described.
Because the patient usually is
anesthetized for biopsy of a lymphadenopathy with a possible diagnosis of
lymphoma, a complete blood count (anemia, platelets) and chest radiography
(mediastinal mass or adenopathies) are mandatory. Exclude the presence of
associated myocarditis (echocardiography).
Large cervical lymph nodes can make
face-mask ventilation, direct laryngoscopy, and tracheal intubation
difficult. The risk of myocarditis is increased, so special anesthetic
consideration must be considered in case of cardiomyopathy-like symptoms.
No known specific pharmacological
implication with this condition. However, patients receiving chronic steroid
therapy may require perioperative supplementation.
Jang YJ, Park KH, Seok HJ: Management of Kikuchi's disease using
glucocorticoid. J Laryngol Otol
Payne JH, Evans M, Gerrard MP: Kikuchi-Fujimoto disease: Rare but important
cause of lymphadenopathy. Acta Paediatr