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A case of migratory lymphadenopathy and cutaneous anergy in an Asian woman
  1. Haider M Al Attia,
  2. Yasser H Al Ahmed,
  3. Afaf El Hag,
  4. Rima N El Abassi
  1. Department of Internal Medicine and Histopathology, Mafraq Hospital, Abu Dhabi, United Arab Emirates
  1. Dr Haider M Al Attia, Mafraq Hospital, PO Box 2951, Abu Dhabi, UAE

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Answers on p 663.

A 40 year old Pakistani woman presented with one month history of fever, night sweats, and progressive and painful swelling in the right side of the neck. She was febrile (temperature 38–40ºC). Right deep cervical, occipital, and jugulodiagastric lymph nodes were palpable, firm, and tender. Save for a moderately severe asymmetrical arthralgia of right wrist, shoulder and proximal interphalangeal joints, the rest of her physical examination was unremarkable. Laboratory data showed, mild leucopenia of 3.6 × 109/l with 64% neutrophils, 26% lymphocytes (no atypical form), and 10% monocytes, erythrocyte sedimentation rate (ESR) 64 mm/hour, and C reactive protein of 105 mg/l (normal <10 mg/l). A skin test for tuberculin reactivity was negative. Results of chest radiography, blood chemistry, urine and stool analysis, throat swab, blood cultures, Venereal Disease Research Laboratory test, test for HIV, heterophil antibodies, and serological studies for salmonella serotypes, brucellosis, toxoplasmosis, Lyme disease, Epstein-Barr virus, cytomegalovirus, human T cell leukaemia virus-1, herpes simplex and hepatitis B and C, as well as serum immunoglobulins were normal or negative. Assays for antinuclear factors, anti-dsDNA, neutrophil cytoplasmic antibodies, and rheumatoid factor were also negative. Computed tomography of the chest and abdomen showed normal findings. Cervical lymph node biopsy showed an intact architecture and a reactive proliferation of histiocytes, transformed lymphocytes of CD8 phenotype and plasma cells, surrounding areas of karyorrhectic necrosis without neutrophils. There were no demonstrable organisms on special stains. A periadenitis was present together with a vasculities with no fibrinoid necrosis present (fig 1). A diagnosis was made and she was treated accordingly. There was rapid resolution of fever and arthralgia within 24 hours, and the lymphadenopathy in two weeks. Follow up at three months was uneventful.

Figure 1

Area of karyorrhectic necrosis (arrow) surrounded by histiocytes (pale zone), lymphoctyes, and plasma cells (dark zone).

After being well for two years, she presented again in June 1998 with a one month history of the same symptoms, but this time there was a non-tender, left supraclaviular, posterior triangle, and jugulodigastric lymphadenopathy. Leucopenia of 3.1 × 109/l with 7% monocytes and ESR of 36 mm/hour were documented. All the tests that had been carried out during her previous admission were repeated. The results showed no abnormal findings. The tuberculin test was again negative. Biopsy of a left deep cervical node showed identical changes to those of the previous biopsy. She was conservatively managed with bed rest and administration of paracetamol. The fever subsided completely after 10 days and lymphadenopathy resolved in two weeks. The patient remains well to date. During the follow up, the tuberculin test was repeatedly found to be positive.

Questions

(1)
What was the overall diagnosis?
(2)
What two unusual features are described in this case?

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