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Revista chilena de pediatría

versión impresa ISSN 0370-4106


VERDUGO L., Patricia; RODRIGUEZ Z., Natalie; TORDECILLA C., Juan  y  SOTO A., Verónica. Pediatric secondary hemophagocytic síndrome: Clinical report of eight cases. Rev. chil. pediatr. [online]. 2005, vol.76, n.4, pp.397-403. ISSN 0370-4106.

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

Palabras clave : Macrophagic activation; hemophagocytosis; intravenous immunoglobulin.

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