Two Months Old Girl with Disseminated Lymphadenopathy and Hepatosplenomegaly

WHAT IS YOUR DIAGNOSIS? A 2-month–old female infant was referred to our center with disseminated lymphadenopathy and hepatosplenomagaly. At birth, she was presented with fever and respiratory distress and underwent sepsis workup . Antibiotic therapy was initiated for her but the fever continued despite administration of broad spectrum antibiotics. Abdominal sonography was performed which revealed paraaortic lymphadenopathy and hepatosplenomegaly. Biopsy of para-aortic lymph nodes showed necrotizing granulomatosis inflammation and polymerase chain reaction (PCR) became positive for Mycobacterium tuberculosis complex. (Mycobacterium tuberculosis, bovis, avium, and BCG). Her parents were evaluated for TB for which the results were negative. She had a history of BCG vaccination at birth. An anti-tuberculosis regimen (INH, RIF, ETB, PZA and Amikacin) was initiated for her. She showed no improvement during two months and therefore, she was referred to our center for further evaluation. On arrival her physical examination showed hepatosplenomegaly and cervical lymphadenopathy. On laboratory tests, CBC and Liver function tests were normal. Tripled gastric aspirate test for M.tuberculosis was negative. Parahillar lymphadenopathy and parenchymal lung involvement were detected in spiral chest CT-scan (Figure 1). Her parents were evaluated for tuberculosis for the second time. Their CXR, chest CT-scan, sputum culture and PPD results were unremarkable. No history of TB was reported in her family and relatives. Her treatment regimen was altered. After 6 months, she became totally symptom free. Lymphadenopathies were diminished and chest CT-scan result was normal (Figure 2). During this period her mother developed diarrhea and a month later she was diagnosed with ascites for which she underwent diagnostic tests.