Abstract

HNL is often misdiagnosed in older children with persistent fever and lymphadenopathy, leading to unnecessary diagnostic tests and evaluations, inappropriate antibiotic administration, and mismanagement. A multidisciplinary team, including primary care providers, rheumatologists, and pathologists, can improve patient outcomes by increasing their awareness of this rare condition. • Histiocytic necrotizing lymphadenitis (HNL) is characterized by fever, leukopenia, and neck lymphadenopathy with unknown etiology. • The lack of neutrophils or eosinophils in the histology, immunohistochemistry results help distinguish HNL from infectious causes.Although HNL is a self-limiting disease, antibiotics and steroid treatments were used inappropriately. • A fever peak ≥ 40 °C was associated with a longer fever duration in HNL patients.Leukopenia presented more frequently in solitary cervical lymph node (LNs) than in extracervical LNs inpatients with prolonged fever. •Steroids are not recommended as a routine treatment, however, in some severe or relapsing cases withpersistent symptoms, prednisolone (5 mg twice a day for 2 days) or other steroids (an equivalent dose ofprednisolone) responded favorably.

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