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Editor,—Kimura’s disease is a chronic inflammatory disorder of unknown aetiology.1 Patients usually present with recurrent painless swellings in the subcutis of the head and neck region, increased serum IgE levels, and peripheral eosinophilia. The disease is described as reactive and data on clonality is absent.
Here we describe a patient with Kimura’s disease involving the orbits. Clonality studies were performed by polymerase chain reaction (PCR) for immunoglobulin heavy chain (IgH), T cell receptor gamma (TCR-γ), and delta (TCR-δ) gene rearrangements.2-4
A 20 year old man presented with a 2 × 3 cm right eyelid swelling in 1986 with normal visual acuity and absence of diplopia. In 1993, he presented with progressive swelling in the right upper eyelid, which subsided with a short course of prednisolone (50 mg/day × 1 week). He was lost to follow up until May 1997 when he developed recurrent swelling of the right upper eyelid. Excision biopsy of the right upper eyelid mass showed changes consistent with Kimura’s disease. DNA was extracted from lacrimal gland biopsy tissue.5 Gene rearrangements for IgH gene, TCRγ, and TCRδ genes were tested by PCR2-4 but no clonal gene rearrangement was identified (Fig 1). In June 1998, he had recurrence of the right upper eyelid mass without any local or systemic symptoms (fever, night sweats, weight loss) and multiple left cervical lymph nodes measuring 1–2 cm in diameter. A complete blood examination showed haemoglobin 14.1 g/l, platelets 282 × 109/l and leucocytes 18.4 × 109/l (differential: eosinophils 3.68 ×109/l, neutrophils 9.2×109/l, lymphocytes 1.5×109/l, and monocytes 4.02×109/l) (normal range: leucocytes 4–11×109/l; eosinophils 0.1–0.4×109/l). The IgE level was 10 328 IU/ml (normal range: <100 IU/ml). IgG, A, and M levels were normal. Urea, creatinine, albumin, and transaminase levels were within normal limits. Serology for HIV was negative. Magnetic resonance imaging (MRI) showed an oval mass in the right lacrimal gland and thickening of the right superior rectus muscle (Fig 2). After 3 weeks of prednisolone (50 mg/day), there was almost complete resolution of the lacrimal mass and IgE level and eosinophil count went down to 2860 IU/ml and 1.2 × 109/l respectively. Computed tomography scan of the abdomen revealed absence of intra-abdominal lymphadenopathy or organomegaly.
Neoplasia is characterised by clonal proliferation of cells and is most often demonstrated in cases of malignant diseases. However, monoclonality has also been demonstrated in some “benign” or “reactive” lymphadenopathy such as angioimmunoblastic lymphadenopathy and Castleman’s disease, both of which are associated with a tendency to aggressive lymphoma.6 7
Kimura’s disease runs an indolent course and has been described as a chronic inflammatory process reactive to some “unknown” stimuli.1 Our patient had a typical clinical presentation with recurrent lacrimal swelling and lymphadenopathy in the head and neck region. It ran an extremely indolent course and, despite the recurring nature of the disease, our patient remained so asymptomatic that he was lost to follow up for years. Interleukin-5 has been shown to be constitutively expressed and explains some of the features of the disease such as eosinophilia and elevated IgE level.8
TCRδ gene has been shown by PCR amplification to be rearranged not only in clonal T cell disorders, but also in 73% of clonal B cell disorder.2 The PCR based methods for the IgH gene rearrangement is positive in 55%–100% of various types of clonal B cell disorders.4 In our patient, the absence of clonal TCR and IgH gene rearrangements is consistent with the reactive nature of the disease. However, despite the relatively high sensitivity of these PCR based techniques to detect clonality, the finding should be confirmed by testing larger numbers of patients and Southern hybridisation with appropriate probes if DNA from fresh tissue is available.
In conclusion, our patient illustrates the typically indolent, recurring nature of the disease with lymphadenopathy and swelling confined to the head and neck region. The failure to demonstrate clonality is consistent with the reactive nature of the entity and the lack of report of malignant lymphoma transformation.