[Hemophagocytic Syndrome: a clinical presentation of systemic lupus erythematosus].
Hemophagocytic Syndrome is a clinical condition characterized by the activation of either macrophages or histiocytes with a prominent hemophagocytosis feature in the bone marrow and other reticuloendothelial systems. It leads to the phagocytosis of erythrocytes, leukocytes, platelets, and their precursors. The presence of hemophagocytosis can be associated to infections, malignancies, autoimmune diseases, drugs and a variety of other medical conditions. We report a case of a previously healthy 23 year-old woman that developed hemophagocytosis at the same time that she fulfilled diagnostic criteria for systemic lupus erythematosus. Lupus-related hemophagocytic syndrome is a rare and potentially fatal entity. It offers significant differential diagnosis challenges and requires urgent therapeutic intervention. There are only few cases reported in the literature. In this article, we briefly reviewed what is currently known about this syndrome. However, much is still needed in order to better understand its causes, all the immunopathogenic mechanisms, as well as its clinical aspects.
Silva
Danilo da Fonseca Reis
Dda F
Medicina da Universidade Federal do Piauí - UFPI, Piauí, Brazil. danilo2002_02@yahoo.com.br
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Fernando Antônio Costa
FA
Arcoverde
Josué da Costa
Jda C
Costa
Loyana Pinheiro
LP
Marques
Igor Denizarde Bacelar
ID
Pinheiro
Gevina da Silva
Gda S
Moreira
Maria do Socorro Teixeira Almeida
Mdo S
D000328:Adult; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology*