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Medicina Interna

versão impressa ISSN 0872-671X

Resumo

MOURA, Teresa Souto et al. Hemophagocytic Syndrome: A Suspect to Have in Mind. Medicina Interna [online]. 2017, vol.24, n.2, pp.128-132. ISSN 0872-671X.

The hemophagocytic syndrome is a rare and potentially fatal condition, characterized by uncontrolled immune system activation, with symptoms and signs of extreme systemic inflammation. As the manifestations are mostly unspecific, its diagnosis requires a high degree of suspicion, in order to implement adequate and timely therapy. The authors present the case of a 19 year-old boy, previously healthy, with a suspected lung infection due to a period of three weeks fever. He presented a sudden increase of liver enzymes and slight pancytopenia, skin rash and hepatosplenomegaly. The elevated values of ferritin and interleukin-2 soluble receptor, as well as the presence of hemophagocytosis in bone marrow, confirmed the diagnosis of hemophagocytic syndrome. After four weeks of dexamethasone, total resolution of symptoms and laboratorial normalization were achieved.

Palavras-chave : Adrenal Cortex Hormones; Ferritins/blood; Lymphohistiocytosis, Hemophagocytic/diagnosis; Lymphohistiocytosis; Hemophagocytic/therapy.

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