Generalized Lymphadenopathy in Infancy; a Case Report
A 7-month-old boy residing in Urmiah, North West Iran, presented with generalized lymphadeno-pathy (LAP) in neck, axilla and groin. He was referred to the Children's Medical Center, the referral pediatric center in Tehran, Iran and admitted to the pediatric infectious disease ward. The patient was a healthy infant till three months ago as his mother noticed a large node on the right side of his neck that had progressive growth and occupied a large portion of cervical area. There was a low grade fever, but no history of upper respiratory tract infection, dyspnea or dysphagia. The child was breastfed and also received supplementary nutrition. Past medical and family history was normal. Clinical examination showed a healthy male infant weighing 8600 gr, length 67 cm and head circumference 45 cm. He had an appearance of bull neck due to bilateral cervical and mandibular LAP. Lymph nodes were discrete, non tender, mobile, matted and about 2 to 6 centimeters in diameter; left axilla and groins also were involved with lymph nodes of almost equal size (). Chest x-ray was normal but the skull X-ray showed some lytic lesions in calvarium. The abdominal sonography was normal. White blood cell count 13140/mm3, neutrophils 55.4%, lymphocytes 39.1%, monocytes 4.2%, eosinophils 1.1%, basophils 0.2%, platelets, 530000/mm3, ESR 56 in the first hour. Hemoglobin 10.3 g/dl. Biochemistry, liver function tests and serologic exams for Epstein-Barr virus, cytomegalovirus, HIV, and Toxoplasma gondii, and also the results of diagnostic studies for tuberculosis including tuberculin skin test (TST) were negative. Primary biopsy of cervical lymph node was done. The histologic diagnosis was compatible with RDD by dilated sinusoids filled with histiocytes, signs of emperipolesis of lymphocytes (). The neoplastic cells expressed positive immunoreactivity for protein S-100, and CD68 and negative immunoreactivity for CD1a that documented the diagnosis.