Revista Brasileira de Reumatologia
Print version ISSN 0482-5004
ARNEZ, Marco Antonio Cuellar; AZEVEDO, Mario Newton Leitão de and BICA, Blanca Elena Rios Gomes. Reactive haemophagocytic syndrome in a systemic lupus erythematosus patient: case report. Rev. Bras. Reumatol. [online]. 2012, vol.52, n.5, pp. 793-795. ISSN 0482-5004. http://dx.doi.org/10.1590/S0482-50042012000500013.
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.
Keywords : reactive macrophage syndrome; hemophagocytic lymphohistiocytosis; systemic lupus erythematosus.