Abstract
Introduction: Secondary hemophagocytic syndrome (SHS) is an uncommon clinical entity with high mortality, characterized by increased macrophagic activity associated to infections, immunodeficiency and neoplastic diseases. The clinical manifestations are persistent fever (> 7 days), hepatosplenomegaly and cytopenia affecting from a single hematological cell line up to pancytopenia. Diagnosis is based on examination of the bone marrow, lymph node or liver showing benign histiocytes with active hemophagocytosis.
Methods: Retrospective analysis of clinical data related to 8 pediatric patients with SHS.
Results: Median age 6 years with the following underlying diseases: hematological cancer (3), autoimmune disease (2) and Down Syndrome (1). Infection was associated with SHS in all patients and the etiology was documented in 4 cases. Intravenous immunoglobulin plus methylprednisolone besides antibiotics were given to 6 patients. Only one patient died.
Conclusion: Early and accurate diagnosis with inmunomodulation therapy was useful to improve outcome and reduce mortality rate in these cases.
This work is licensed under a Creative Commons Attribution 4.0 International License.
Copyright (c) 2005 Revista Chilena de Pediatría