A 9month-old-boy with atypical hemophagocytic lymphohistiocytosis

Monia Ouederni, MONIA BEN KHALED, Samia Rekaya, Ilhem Ben Fraj, Fethi Mellouli, Mohamed Bejaoui
  • Monia Ouederni
    Faculty of Medicine, University of Tunis El Manar, Tunis, Tunisia. Bone marrow transplantation center Tunis, Tunisia | moniahasan@yahoo.fr
  • MONIA BEN KHALED
    Faculty of Medicine, University of Tunis El Manar Bone marrow transplantation center Tunis, Tunisia
  • Samia Rekaya
    Faculty of Medicine, University of Tunis El Manar Bone marrow transplantation center Tunis, Tunisia
  • Ilhem Ben Fraj
    Faculty of Medicine, University of Tunis El Manar Bone marrow transplantation center Tunis, Tunisia
  • Fethi Mellouli
    Faculty of Medicine, University of Tunis El Manar Bone marrow transplantation center Tunis, Tunisia
  • Mohamed Bejaoui
    Faculty of Medicine, University of Tunis El Manar Bone marrow transplantation center Tunis,

Abstract

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.

Keywords

Anemia, Neutropenia, , thrombocytopenia, hyperfeeeritinemia, Haemophagocytic lymphohistiocytosis

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Submitted: 2017-06-19 05:57:51
Published: 2017-10-16 00:00:00
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