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An unusual case of pyrexia of unknown origin with cervical lymphadenopathy
  1. P Wurma,
  2. G Townsona,
  3. I Lauderb,
  4. A C Wicksa
  1. aDepartment of Gastroenterology, Leicester General Hospital, bDepartment of Pathology, Leicester Royal Infirmary
  1. Dr P Wurm, Department of Gastroenterology, Leicester Royal Infirmary, PO Box 65, Leicester LE1 5WW, UK (email: pwurm{at}uhl.trent.nhs.uk)

Abstract

Kikuchi's disease is usually a self limiting illness characterised by pyrexia, neutropenia, and cervical lymphadenopathy particularly in young women of Asian descent. This often leads to an initial misdiagnosis of lymphoma. A case of a young Asian woman who presented with pyrexia of unknown origin is described.

  • pyrexia of unknown origin
  • cervical lymphadenopathy

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Case report

A 19 year old south Asian women was admitted with a six week history of fever, malaise, anorexia, and cervical lymphadenopathy. She had been well until onset of her current illness. She was on no medication and had no contacts with tuberculosis.

She was born in India but had moved to the UK at the age of 9 years. Foreign travel included a brief return to her native country 10 years previously and a trip to the United States two years before presentation.

Clinical examination revealed a young women who appeared well; her temperature was 39ºC. The main findings were several tender mobile posterior cervical lymph nodes of which one was large. There were no lymph nodes elsewhere and she had no splenomegaly. The rest of the clinical examination was unremarkable.

Investigations revealed a low white cell count at 2.7 × 109/l with a neutrophil count of 1.3 × 109/l, a raised plasma viscosity of 1.99 cp (normal range 1.5–1.72). A biochemical profile, chest radiography, and abdominal ultrasound gave normal results.

The clinical impression was that this was likely to be tuberculous as she was Asian, or it could possibly be a lymphoma. Her clinical course was of a swinging intermittent pyrexia of up to 40.5ºC. Blood and urine cultures, thin and thick films for malaria parasites, cytomegalovirus, Epstein-Barr virus, and HIV titres were all negative.

Tests for antinuclear factor, double stranded DNA, and antineutrophil cytoplasmic antibodies were negative. Thyroid microsomal antibody titres were positive at 1/400 and T cell analysis showed severe T lymphopenia of CD4, CD8, and NK populations with T cell activation.

Later in her illness she had computed tomography of her chest, abdomen, and pelvis which was unremarkable, although minimal splenic enlargement was noted. Bone marrow aspiration and a trephine biopsy did not show any evidence of lymphoma or non-caseating granulomas.

The large posterior cervical lymph node was excised. Microscopic examination using an antibody against CD 68 showed numerous darkly staining histiocytes, many of them containing cell fragments. There was necrosis and apoptosis and immature lymphoid cells in keeping with a diagnosis of Kikuchi's disease. Kikuchi's disease was originally described in the Far East in 1972 and over the last decade has become increasingly recognised in the Western world.

Learning points

  • Kikutchi's disease should be considered in patients presenting with pyrexia, neutropenia, and cervical lymphadenopathy

  • It usually affects young female Asian patients

  • The disease is self limiting with a good prognosis and most patients appear to recover within a few weeks without any serious sequelae

Antibiotic treatment was withdrawn. Her temperature settled after three weeks, but she continued to remain neutropenic for six weeks from the beginning of the illness. When reviewed in outpatients four weeks after discharge from hospital she was well and all her lymph nodes had disappeared.

Discussion

The combination of pyrexia, neutropenia, and cervical lymphadenopathy particularly in young Asian women often leads to an initially wrong diagnosis of tuberculosis or even lymphoma.1 A massive release of cytokines, particularly interferon gamma and tumour necrosis factor, account for these symptoms and the necrosis.

Associations with systemic lupus erthymatosus and other autoimmune disorders (mixed connective tissue disease, Hashimoto's thyroiditis) have been proposed.2 3 There is also anecdotal evidence of postviral disease.4

The disease is self limiting with a good prognosis and most patients appear to recover within a few weeks without any serious sequelae. The importance of this case is that although it is well recognised by pathologists, rheumatologists,2 and ear, nose, and throat surgeons,5 it is not a particularly well known entity as far as general physicians in the UK are concerned. This was borne out by a straw poll of physicians in this hospital who were not aware of this condition clinically. There have been few reports of Kikuchi's disease occurring in the UK, to our knowledge none by a general physician. This hopefully will make people, particularly where there is a large Asian population such as in Leicester, more aware of this benign condition which is readily diagnosed on lymph node biopsy.

References

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