Mediterranean Journal of Hematology and Infectious Diseases (Oct 2017)

A 9month-old-boy with atypical hemophagocytic lymphohistiocytosis

  • Monia Ouederni,
  • MONIA BEN KHALED,
  • Samia Rekaya,
  • Ilhem Ben Fraj,
  • Fethi Mellouli,
  • Mohamed Bejaoui

DOI
https://doi.org/10.4084/mjhid.2017.57
Journal volume & issue
Vol. 9, no. 1
pp. e2017057 – e2017057

Abstract

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Hemophagocytic lymphohistiocytosis is a life-threatening hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and histiocytes. Often, Hemophagocytic lymphohistiocytosis is an acquired syndrome. We report a case of a 9 month-old-boy presented with hepatosplenomegaly, severe anemia requiring repeated transfusions, thrombocytopenia, hypertriglyceridemia and very high hyperferritinemia. These clinical features of Hemophagocytic lymphohistiocytosis prompted a wide infectious and auto-immune request to be performed. After a wide diagnostic workup, he was referred to the immune hematologic unit, for hemophagocytic lymphohistiocytosis suspicion with unknown cause.

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