A 9month-old-boy with atypical hemophagocytic lymphohistiocytosis

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Monia Ouederni
Samia Rekaya
Ilhem Ben Fraj
Fethi Mellouli
Mohamed Bejaoui


Anemia, Neutropenia, , thrombocytopenia, hyperfeeeritinemia, Haemophagocytic lymphohistiocytosis


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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