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At a glance

It is a benign, histiocytic necrotizing lymphadenitis usually affecting young women. It is often mistaken for lymphoma or Hodgkin disease. It is characterized by fever, large lymphadenopathy, skin rashes, and throbbing headaches. Other features may include hepatosplenomegaly and meningitis-like symptoms. Extreme fatigue is reported. The disease resolves spontaneously within 2 to 3 months, and relapse is uncommon.

Synonym

Histiocytic necrotizing lymphadenitis.

History

It was first described in Japan in 1972 by Dr Masahiro Kikuchi (1935-2012) and later expanded by Y. Fujimoto, both Japanese physicians.

Incidence

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 described.

Pathophysiology

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.

Diagnosis

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 infiltrate.

Clinical aspects

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.

Precautions before anesthesia

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).

Anesthetic considerations

Large cervical lymph nodes can make face-mask ventilation, direct laryngoscopy, and tracheal intubation difficult. The risk of upper airway obstruction was reported during anesthesia possibly due to the presence of large lymphadenopathy. The risk of myocarditis is increased, so special anesthetic consideration must be considered in case of cardiomyopathy-like symptoms.

Pharmacological implications

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