A 1/5 year old boy was hospitalized for lymphadenopathy and low grade fever for 1 week. His weight was 7 kg. In physical examination, multiple lymphadenopathy in cervical and inguinal areas together with hepatosplenomegaly were diagnosed. He was the second child of non relative parents. The first child of family died because of respiratory infection at the age of 6 months old. Results of Para clinic evaluation were reported as below:
WBC /9000, Hb/8/5. Plt /51000, ESR/92, CRP/Neg, LDH/normal , TG/277, uric acid /3, AST/36, ALT/14, Alp/306, Total bilirubin /0.4, Total protein/8.7 Alb/4.6, Urea/ 27, Cr/0.5, Urine analysis. Stool examination, Bone marrow aspiration and CXR were all normal.
Abdominal sonography / enlarged liver and spleen with normal echo, liver biopsy/Mononuclear cell infiltration in portal tracts, in addition to expanded fibrotic spaces and mild to moderate piecemeal necrosis. Immunologic and virology assessment were requested, but his parents refused to do it, and as a result he was discharged. In an outpatient visit, in 3 months later, he improved significantly, but thrombocytopenia had been persisted. They did not return for follow up till nine months later. One year after the first visit, the boy was hospitalized again with FUO and protracted cough for 20 days. In physical examination weight was 9 kg, Height/80 cm and head circumference/ 47cm; all of them were below 3 percentile. Hepatosplenomegaly and cervical lymphadenopathy were apparent. We thought about viral infections, TB, salmonellosis, malignancy, immune deficiency and collagen vascular diseases for this patient. Para clinic data have shown as following: WBC/8000, Hb/8.4, plt/147000, ESR/97, CRP/negative, TG/353, Total protein /9.8.AST/31, ALT/26, ALP/286.Alb/3.2, Bil/0.3, LDH/838, Retic/2%, Coombs/ negative and EBV( Ig M and IgG) was found to be Positive. Abdominal sonography showed enlargement of liver and spleen but bone marrow aspiration was normal with no acid fast bacillus , PPD test / negative and lymph node biopsy showed reactive lymphadenitis , flow cytometry / CD4: 26.8,CD3: 75,CD16: 10.2,CD19: 4.5,CD56 : 9 ,CD18: 95. In ophthalmologic consultation, bilateral optic neuritis was seen, and brain MRI showed thrombosis of right transverse sinus. Echocardiography was normal, but antipospholipid antibodies were present and decreased level of protein S and factor 5 Leiden and increased level of anti thrombin III was detected. HIV antibody was reported positive in the patient and his parents. By reviewing the family history, it was apparent that the patient s father had been drug abuser from two years before. Therapy with Warfarin and Cotrimoxazole was prescribed for the patient and they were referred to the special center for further treatment.