title: Reactive haemophagocytic syndrome in a systemic lupus erythematosus patient: case report.
abstract: The macrophagic syndrome or reactive haemophagocytic syndrome (RHS) is a complication resulting from systemic inflammatory diseases and may also be related to malign neoplasias, immunodeficiencies and to a variety of infections caused by virus, bacteria, and fungus. It is characterized by an excessive activation of macrophages and histiocytes along with intense hemophagocytosis in bone marrow and reticulum-endothelial system, causing the phagocytosis of erythrocytes, leukocytes, platelets, and their precursors. The clinical manifestations are fever, hepatosplenomegaly, lymphadenomegalies, neurological involvement, variable degrees of cytopenias, hyperferritinemia, liver disorders, intravascular coagulation, and multiple organs failure. We report a rare case of recurrent RHS complication in a systemic lupus erythematosus male patient after two years. Although extremely rare it has evolved with an improvement after a pulse methilprednisolone and cyclophosphamide therapy.
authors.affiliation: Serviço de Reumatologia, Universidade Federal do Rio de Janeiro, Brazil. marcoarnez@hotmail.com
authors.forename: Marco Antonio Cuellar
authors.initials: MA
authors.lastname: Arnez
authors.forename: Mario Newton Leitão
authors.initials: MN
authors.lastname: de Azevedo
authors.forename: Blanca Elena Rios Gomes
authors.initials: BE
authors.lastname: Bica
mesh_terms: D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology*; D008297:Male; D008875:Middle Aged