Hemophagocytic Syndrome: Report of 3 cases

Abstract

Hemophagocytic Síndrome (HS) is a hiperinflammatory syndrome that results from an uncontrolled and ineffective immune response associated to fever, hepatosplenomegaly, hepatic failure, pancytopenia, adenopathies and coagulopathy, also featuring lymphohistiocytic tissue proliferation and hemophagocytosis. They are classified as primary and secondary, with the latter ones associated to infectious, neoplastic, autoimmune and pharmacological processes. Diagnosis is difficult in pediatriatic population, given the similiarity to other diseases, the cardinal signs are fever, hepatosplenomegaly and cytopenias. Diagnostic criteria are dictated by the Histocytosis Society in 2004 and are presented in Table 3. Although they are not listed in the diagnostic criteria, almost all of these patients have hepatitis manifested by elevated liver enzymes. It is relevant to perform bone marrow studies in order to define hemophagocytosis, but in almost 20% of cases in early stages it is difficult to achieve. Treatment should begin immediately and the patient should be referred urgently to a pediatric hemato-oncology service, because delay in treatment can lead to irreversible organ failure.