Haemophagocytic lymphohistiocitosis - a case report in infant

Background: The purpose of this case report is to alert for a serious disease, potentially lethal, often confused with sepsis, however, it is an auto inflammatory disease, with massive activation of macrophages and consequent tissue destruction.

Methods: Review of electronic medical record

Results: We report a case of an infant, 1 year and 8 months old, male, that was in intensive care unit for five months with initial diagnosis of acute diarrhea, dehydration and hemolytic uremic syndrome.

The infant developed fever, hepatosplenomegaly, cutaneous rash, bicytopenia, elevated serum ferritin, liver enzyme abnormalities, encephalitis, acute renal failure and serum hypogammaglobulinemia.

The diagnosis of hemophagocytic lymphohistiocytosis was suspected and confirmed with biopsy of bone marrow with evidence of hemophagocytosis.
Chemotherapy was instituted promptly with etoposide, dexamethasone and intravenous human immunoglobulin (immunomodulation dosis), with a favorable evolution and discharge from the intensive care unit.

Conclusions: We call attention to the clinical and laboratory diagnosis of hemophagocytic histiocytosis which is a potentially lethal disease if not diagnosed early.