| Literature DB >> 23732203 |
Carolina V Mahuad1, Gonzalo M Garate, María de Los Ángeles Vicente Reparaz, Claudia Casali, Mercedes Del Olmo, Alberto Bolgiani.
Abstract
The hemophagocytic syndrome represents an infrequent, occasionally misdiagnosed and usually fatal heterogeneous entity. Infections, drugs, autoimmune diseases and cancer are often triggers of the secondary hemophagocytic syndrome. Its physiopathogenic mechanism is explained by an impaired and inefficacious function of the NK and T cytotoxic cells that leads to an ineffective and uncontrolled immune response, inducing cellular damage, multiorganic failure with macrophage proliferation and hemophagocytosis. The main objective of the different therapeutic options, commonly combinations of steroids and chemotherapy, is the suppression of the uncontrolled immune response. Occasionally, the clinical condition of some patients represents a contraindication for intensive treatment. We report a case of a severely burned patient that fulfilled the revised criteria for the hemophagocytic syndrome and was successfully treated with the combination of intravenous immunoglobulins and steroids.Entities:
Keywords: hemophagocytic lymphohistiocytosis; hemophagocytosis; intravenous immunoglobulins
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Year: 2013 PMID: 23732203
Source DB: PubMed Journal: Medicina (B Aires) ISSN: 0025-7680 Impact factor: 0.653