Kikuchi's disease in a 9-year-old girl: case report

Abstract

A 9-year-old girl was admitted with a 2month history of continuous, moderate-tohigh-grade fever, weight loss and gradual onset of pallor. There was no history of cough, diarrhoea, urinary symptoms, rash or arthralgia. On examination, she was emaciated, febrile and pale. There were multiple, matted, tender cervical lymph notes of firm consistency, predominantly on the right side. Axillary and inguinal lymph nodes were not palpable. There was hepatomegaly of 3 cms. The spleen was not palpable. Hb was 6 g/dl and blood film showed a hypochromic, microcytic picture; WBCs were 2.1610/L, neutrophil count 1.2610/L and platelets 253610/L. ESR was 121 mm in the 1st hour. Cultures of blood and urine and throat swabs were sterile. Serology for cytomegalovirus (CMV) was positive, both IgG and IgM, and negative for Epstein–Barr virus. However, PCR for CMV in blood and urine was negative. A Mantoux test and PCR for mycobacteria in blood were also negative. Anti-nuclear antibody, double-stranded DNA and anti-nuclear cytoplasmic antibody were all below the reference range. Bone marrow aspirate revealed normal haematopoiesis, and trephine was normal. Chest X-ray and ultrasound of the abdomen were normal. However, CT scan of the chest showed enlarged axillary lymph nodes with dense nodules within the parenchyma of the right lung but there were no mediastinal lymphadenopathy. Gallium and bone scans were normal. Lymph node histology demonstrated multiple areas of necrosis with preservation of only a few irregular follicles. The areas of necrosis were situated peripherally and were of irregular shape and bordered by proliferating histiocytes. Within the areas of necrosis there was abundant nuclear debris. The nucleus of the histiocytes appeared to be crescent-shaped as a result of being pushed away by abundant pink cytoplasm. Polymorphonuclear leucocytes were conspicuously absent and there were no granulomas. The findings were compatible with necrotising lymphadenitis.