Hemophagocytic Lymphohistiocytosis (HLH) in Children Presenting as Liver Disease

Nomisha Amin1, Ira Shah1, Sushmita Bhatnagar1

  • 1Pediatric Liver Clinic, B.J. Wadia Hospital for Children, Mumbai, India.

Hemophagocytic lymphohistiocytosis (HLH) is a rare acute hyperinflammatory condition presenting with high fever, pancytopenia, splenomegaly with the pathologic finding of hemophagocytic lymphohistiocytosis in bone marrow and other tissues. Predominant hepatic manifestations at presentation are rare. We report a series of three cases which showcase the spectrum of liver disease as presentation in hemophagocytic lymphohistiocytosis.

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