Liver Abscesses and Hyper IgM Syndrome
A 5-year-old boy presented with recurrent fever for 1.5 months and intermittent pain in right hypochondrium along with vomiting for 1 month. On examination, he had tender hepatomegaly and pallor. Investigations showed multiple small hypodense lesions in the liver suggestive of microabscesses. His investigations showed hemoglobin 8.8 gm/dL, WBC count 17,400/mm3, platelet count 438,000/mm3, bilirubin 0.8 mg/dL, SGOT 102 IU/L, SGPT 68 IU/L, total proteins 5.5 gm/dL, and albumin 2.4 gm/dL. Stool showed cysts of E. histolytica and blood culture did not grow any organism. HIV ELISA was negative. He was treated with IV piperacillin/metronidazole and oral chloroquine for 10 days but had no response. He subsequently had a drop in hemoglobin level along with leukocytosis (34,500/mm3) and thrombocytopenia for which he received 5 units of blood transfusion and antibiotics were changed to vancomycin and ceftazidime. He also developed ascitis and ascitic fluid showed 1920 cells/mm3 (80% polymorphs, 20% lymphocytes). Urine examination showed presence of fungal hyphae and child was treated with amphotericin B. In view of severe infections, he was tested for immunodeficiencies. His HIV ELISA was negative and serum immunoglobulins showed low serum IgG (530 mg/dL (normal = 971-1746 mg/dL)), normal IgA (104 mg/dL (normal = 75-178 mg/dL)), and high serum IgM (183 mg/dL (normal = 66-153 mg/dL)). Nitroblue tetrazolium (NBT) test was normal. Patient responded to above therapy and is currently asymptomatic.