HEPATIC ENCEPHALOPATHY - A PRESENTATION OF HEMOPHAGOCYTOSIS
Dr. Ira Shah *
In charge, Pediatric Hepatobiliary, Department of Pediatric Hepatobiliary Clinic, B. J. Wadia Hospital for Children, Parel, Mumbai. *
Abstract: Hemophagocytosis is the consumption of hemopoietic cells by benign (non-malignant) phagocytic cells (activated monocytes / macrophages / histiocytes). Clinically the syndrome is characterized by fever, hepato-splenomegaly, hepatic and other organ failure, and cytopenias or pancytopenia. The neurologic symptoms are extremely variable, ranging from irritability, to convulsions, cranial nerve palsies, peripheral neuropathy, ataxia, hemiplegia, and unconsciousness.

Case Report: A one and a half year old boy born of non-consanguineous marriage presented with fever 15 days back associated with generalized tonic clonic convulsion following which the child lost all previously achieved milestones. On examination, he had hepatosplenomegaly with icterus and constant upward gaze. Investigations showed pancytopenia with low Reticulocyte count. CSF analysis was normal. CT brain revealed multiple areas of hypomyelination. Ultrasound of abdomen showed lymph nodes at porta hepatis with hepatosplenomegaly. Liver function tests showed elevated bilirubin (3.0 mg/dl) with elevated serum transaminases (SGOT = 250 IU/L, SGPT = 175 IU/L) with hypoalbuminemia (2.1 mg/dl). Renal function tests were normal. In view of pancytopenia, a bone marrow examination was done that showed hemophagocytosis. His viral markers in form of Dengue IgM, Leptospira IgM, HBsAg and HIV ELISA were negative. His Herpes simplex IgM, Epstein Barr virus and cytomegalovirus studies could not be done due to unaffordability. He had multiple intracranial bleeds and finally succumbed to his illness.
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