TY - JOUR
T1 - Contemporary diagnostic methods for hemophagocytic lymphohistiocytic disorders
AU - Johnson, Theodore S.
AU - Villanueva, Joyce
AU - Filipovich, Alexandra H.
AU - Marsh, Rebecca A.
AU - Bleesing, Jack J.
PY - 2011/2/1
Y1 - 2011/2/1
N2 - 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.
AB - 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.
KW - HLH
KW - Hemophagocytic lymphohistiocytosis
KW - Hemophagocytosis
KW - Histiocytosis
KW - Macrophage
KW - T cell
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UR - http://www.scopus.com/inward/citedby.url?scp=78650811211&partnerID=8YFLogxK
U2 - 10.1016/j.jim.2010.11.006
DO - 10.1016/j.jim.2010.11.006
M3 - Review article
C2 - 21110979
AN - SCOPUS:78650811211
SN - 0022-1759
VL - 364
SP - 1
EP - 13
JO - Journal of Immunological Methods
JF - Journal of Immunological Methods
IS - 1-2
ER -