A 10-year-old girl with cervical lymphadenopathy, fever, and cytopenia

A 10-year-old, previously healthy female of Chinese descent presented to hospital following 6 days of fever and a 2-week history of right-sided cervical lymphadenopathy. Associated symptoms included a rash, fatigue, anorexia, and epistaxis. On examination, she was febrile and tachycardic. Her physical exam was significant for a 2-cm, tender, right-sided cervical lymph node, a diffuse erythematous maculopapular rash, palmar erythema, and one buccal oral ulcer. Investigations were significant for leukopenia (2.78×10^9/L), neutropenia (0.56×10^9/L), thrombocytopenia (73×10^9/L), and increased markers of systemic inflammation. She had a normal chest x-ray, and an abdominal ultrasound demonstrated mild hepatosplenomegaly. Piperacillin-Tazobactam was empirically started. By day two of admission, she had pancytopenia. After discussion with the rheumatologist, she was treated with intravenous immunoglobulin for possible atypical Kawasaki disease. Six days later, her cervical lymph node had decreased in size and tenderness, and she was afebrile. She was discharged home with the diagnosis of a probable viral illness. Follow-up testing demonstrated normalization of her blood work. She returned to hospital two months later with a one week history of fever and a 2-cm, tender left-sided cervical lymph node. A lymph node biopsy revealed the diagnosis. The pathology from our patient’s lymph node biopsy demonstrated necrotizing lymphadenitis of Kikuchi-Fujimoto disease (KFD). KFD, also known as histiocytic necrotizing lymphadenitis, most often presents as cervical lymphadenopathy with fever. It is a benign, self-limited disease, most commonly described in young women of Asian descent (1). KFD has been reported across a wide age range, from 19 months to 75 years old, though it primarily affects young women in their third decade of life (2). However, KFD is becoming increasingly recognized in the paediatric population (1). In contrast to adults, recent studies have reported a male predominance in younger children, reporting male to female ratios of up to 1.9:1 (3,4). The most commonly reported features of KFD are unilateral posterior cervical lymphadenopathy (typically 2 to 3 cm) and fever. Fatigue, anorexia, weight loss, night sweats, rash, and arthalgias are also common symptoms (1). Laboratory investigation typically shows inflammatory changes, including increased erythrocyte sedimentation rate (ESR) and c-reactive protein (CRP). Lactate dehydrogenase (LDH) and hepatic transaminases are often elevated. Leukopenia, and less commonly, thrombocytopenia and anemia may be present, as seen in our patient (5). Imaging studies are typically nonspecific (6). See Table 1 for the most common clinical findings of KFD in paediatric patients based on two recent studies (3,7). Common features of Kikuchi-Fujimoto disease in the paediatric population (3,7) Common features of Kikuchi-Fujimoto disease in the paediatric population (3,7) Currently, the diagnosis of KFD is confirmed by the histologic findings from lymph node biopsy. However, the diagnosis of KFD is challenging given the nonspecific presentation and lack of a specific and noninvasive diagnostic test. In addition, the typical constellation of clinical symptoms and laboratory findings seen in KFD may also be found in other life-threatening conditions (1,2,5). As such, patients diagnosed with KFD will often have undergone multiple invasive procedures to rule out other diagnoses, including bone marrow and lymph node biopsies. Reasons to consider biopsy of a cervical lymph node include the following: persistence of the lymphadenopathy beyond 4 weeks, size greater than 2 cm, matting and limited mobility on palpation, signs or symptoms suggestive of malignancy, and suspicion for an unusual infection that may require specific therapy (i.e., mycobacterial infection) (8,9). In our case, the patient underwent biopsy because of the recurrence of her disease, concerning laboratory findings, and need to rule out malignancy (8). Most cases of KFD resolve spontaneously within 1 to 4 months. The reported recurrence rate varies, with rates as high as 21% (6). However, all patients with KFD should be monitored for the development of autoimmune diseases, specifically systemic lupus erythematous (SLE). Our patient is currently being investigated for SLE given the development of a new photosensitive rash, oral ulcers, a positive ANA and dsDNA. The development of SLE has been reported in up to 13% of patients diagnosed with KFD. Their clinical presentations overlap significantly and KFD may precede, coincide, or follow the diagnosis of SLE. However, the exact relationship between KFD and SLE remains unclear and complex. Some clinicians have proposed that cases of KFD may actually represent lupus lymphadenitis, whereas others suggest that SLE and KFD may co-exist in patients predisposed to autoimmune diseases (10,11). No clinical treatment guidelines exist for KFD. Treatment has been primarily based on case reports and clinical experience. Observation pending spontaneous regression with supportive measures (e.g., nonsteroidal anti-inflammatory drugs) is a common option. For persistent or severe cases, oral corticosteroids are often used, though the ideal dose and duration of treatment has not been determined (1–4). More recently, alternative treatments have shown some success. For example, given hydroxychloroquine’s role in SLE, it has been proposed as a potential treatment for KFD (1,12,13). Similarly, practitioners have tried intravenous immunoglobulin given its immunomodulatory role in many other inflammatory conditions. Success has been reported in case reports only (1) and it is possible that it may have had some therapeutic effect in our patient. KFD should be considered in the paediatrician’s differential diagnosis for children presenting with prolonged cervical lymphadenopathy with fever. Other associated clinical findings may include rash, constitutional symptoms, arthralgias, elevated ESR, CRP, LDH, and leukopenia. Less commonly, but as seen in our case, thrombocytopenia may be present and KFD may recur after a period without symptoms. It is important to monitor patients diagnosed with KFD due to the increased risk of autoimmune conditions, specifically SLE. Informed consent: Informed consent has been obtained for publication of this case. Funding: There are no funders to report for this submission. Potential Conflicts of Interest: All authors: No reported conflicts of interest. All authors have submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest. Conflicts that the editors consider relevant to the content of the manuscript have been disclosed.

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