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Contemporary diagnostic methods for hemophagocytic lymphohistiocytic disorders

2010; Elsevier BV; Volume: 364; Issue: 1-2 Linguagem: Inglês

10.1016/j.jim.2010.11.006

1872-7905

Theodore S. Johnson, Joyce Villanueva, Alexandra H. Filipovich, Rebecca Marsh, Jack Bleesing,

Parvovirus B19 Infection Studies

Hemophagocytic lymphohistiocytosis is a life-threatening multi-system hyperinflammatory disorder characterized by dysfunctional cytolytic lymphocyte responses, hypercytokinemia, and widespread lymphohistiocytic tissue infiltration and destruction. Diagnosis and definitive therapy are often delayed as clinical efforts are directed toward treatment of presumed overwhelming infection. Sporadic cases occur in association with underlying immune dysfunction related to autoimmune disease, malignancy, or severe infection. However, familial cases predominate with remarkable associations between underlying genetic defects and dysregulation of immune responses. Here, we review the genetic and immunologic basis of contemporary diagnostic methods for hemophagocytic lymphohistiocytosis.