Síndrome hemofagocítico secundario: reporte de 5 casos / Secondary hemophagocytic syndrome: report of 5 cases

ABSTRACT

Secondary hemophagocytic syndrome (HFS) is an uncommon entity with a high mortality rate in adults, if no therapy is given. It is characterized by a severehipercytokinemia due to a highly stimulated but ineffective immune system. Theprincipal causes are infections, malignancy or autoimmune diseases. It appears asa serious illness, similar to a multiorgan failure. Treatment is not well defined. Wereport five patients with HFS, aged 17 to 51 years (three females). The etiology wasonco-hematological in three patients. In two patients, the diagnosis was performedduring necropsy. One case was due to cytomegalovirus (CMV) infection in a hepatictransplant patient and the other, due to parenteral lipid administration. All presentedfever, cytopenia, hepatosplenomegaly and hemophagocytosis. Four of them requiredadmission in an Intensive Care Unit. All received different treatment modalities. Onlyone survived. Median survival time was 75 days. In conclusion, HFS has differentetiologies and a high mortality in adults.