[Secondary hemophagocytic syndrome: report of 5 cases].
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 severe hipercytokinemia due to a highly stimulated but ineffective immune system. The principal causes are infections, malignancy or autoimmune diseases. It appears as a serious illness, similar to a multiorgan failure. Treatment is not well defined. We report five patients with HFS, aged 17 to 51 years (three females). The etiology was onco-hematological in three patients. In two patients, the diagnosis was performed during necropsy. One case was due to cytomegalovirus (CMV) infection in a hepatic transplant patient and the other, due to parenteral lipid administration. All presented fever, cytopenia, hepatosplenomegaly and hemophagocytosis. Four of them required admission in an Intensive Care Unit. All received different treatment modalities. Only one survived. Median survival time was 75 days. In conclusion, HFS has different etiologies and a high mortality in adults.
Camila
C
Peña
Ximena
X
Valladares
María Elena
ME
Cabrera
D000293:Adolescent; D000328:Adult; D003586:Cytomegalovirus Infections / Q000150:complications*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007362:Intensive Care Units; D008055:Lipids / Q000009:adverse effects*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology*; D008223:Lymphoma / Q000150:complications*; D008297:Male; D008875:Middle Aged; D057947:Parenteral Nutrition Solutions / Q000009:adverse effects*; D055815:Young Adult
D008055:Lipids; D057947:Parenteral Nutrition Solutions