Enigmatic Kikuchi-Fujimoto Disease: A Comprehensive Review

Xavier Bosch, MD; Antonio Guilabert, MD; Rosa Miquel, MD; Elias Campo, MD

Disclosures

Am J Clin Pathol. 2004;122(1) 

In This Article

Clinical Manifestations and Laboratory Evaluation

Clinical Manifestations

The onset of KFD is acute or subacute, evolving during a period of 2 to 3 weeks. Cervical lymphadenopathy is present in 56% to 98% of cases, more commonly consisting of tender lymph nodes involving the posterior cervical triangle (88.5%), generally unilateral (88.5%). Lymph node size ranges from 0.5 to 4 cm (93.4%), and occasionally, lymph nodes are larger than 6 cm. Painful lymphadenopathy is seen in up to 59% of patients. Generalized lymphadenopathy has been reported in 1% to 22% of cases.[1,2,16,17,18,22] Involvement of mediastinal, peritoneal, and retroperitoneal regions is uncommon.[7] In addition to lymphadenopathy, 30% to 50% of patients with KFD might have fever, usually low-grade, associated with upper respiratory symptoms. Less frequent symptoms include weight loss, nausea, vomiting, sore throat, and night sweats.[1,2,10,19,22] It should be mentioned that systemic symptoms are found more frequently when extranodal involvement is present.[19]

Laboratory Evaluation

The results of a wide range of laboratory studies usually are normal in KFD. Some patients have anemia and a slight elevation of the erythrocyte sedimentation rate. Mild leukopenia has been observed in 25% to 58% of patients, whereas leukocytosis is found in 2% to 5% of cases. Moreover, 25% to 31% of patients have atypical peripheral blood lymphocytes,[1,2,16,17,18,22] which might support the aforementioned speculated viral cause. The mechanism of granulocytopenia in a patient with KFD has been studied using an in vitro culture system.[1] The number of granulocyte precursor cells (colony forming units in culture [CFU-C]) in the bone marrow was found to be decreased. While T lymphocytes from the patient had no significant suppressor effect on the CFU-C, the patient's serum blocked CFU-C in vitro.[1] The authors proposed that one or more inhibitory factors might cause granulocytopenia in patients with KFD. Finally, serum levels of lactate dehydrogenase and aminotransferases are increased in some patients with KFD.[30] In 2002 we saw a patient with typical clinical and histopathologic findings of KFD who, in addition to anemia, leukopenia, and an increased serum lactate dehydrogenase level, had moderate thrombocytopenia with a normal bone marrow examination (unpublished data).

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