HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.
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HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. / Henter, Jan-Inge; Horne, Annacarin; Aricó, Maurizio; Egeler, R Maarten; Filipovich, Alexandra H; Imashuku, Shinsaku; Ladisch, Stephan; McClain, Ken; Webb, David; Winiarski, Jacek; Janka-Schaub, Gritta.
in: PEDIATR BLOOD CANCER, Jahrgang 48, Nr. 2, 2, 2007, S. 124-131.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.
AU - Henter, Jan-Inge
AU - Horne, Annacarin
AU - Aricó, Maurizio
AU - Egeler, R Maarten
AU - Filipovich, Alexandra H
AU - Imashuku, Shinsaku
AU - Ladisch, Stephan
AU - McClain, Ken
AU - Webb, David
AU - Winiarski, Jacek
AU - Janka-Schaub, Gritta
PY - 2007
Y1 - 2007
N2 - In HLH-94, the first prospective international treatment study for hemophagocytic lymphohistiocytosis (HLH), diagnosis was based on five criteria (fever, splenomegaly, bicytopenia, hypertriglyceridemia and/or hypofibrinogenemia, and hemophagocytosis). In HLH-2004 three additional criteria are introduced; low/absent NK-cell-activity, hyperferritinemia, and high-soluble interleukin-2-receptor levels. Altogether five of these eight criteria must be fulfilled, unless family history or molecular diagnosis is consistent with HLH. HLH-2004 chemo-immunotherapy includes etoposide, dexamethasone, cyclosporine A upfront and, in selected patients, intrathecal therapy with methotrexate and corticosteroids. Subsequent hematopoietic stem cell transplantation (HSCT) is recommended for patients with familial disease or molecular diagnosis, and patients with severe and persistent, or reactivated, disease. In order to hopefully further improve diagnosis, therapy and biological understanding, participation in HLH studies is encouraged.
AB - In HLH-94, the first prospective international treatment study for hemophagocytic lymphohistiocytosis (HLH), diagnosis was based on five criteria (fever, splenomegaly, bicytopenia, hypertriglyceridemia and/or hypofibrinogenemia, and hemophagocytosis). In HLH-2004 three additional criteria are introduced; low/absent NK-cell-activity, hyperferritinemia, and high-soluble interleukin-2-receptor levels. Altogether five of these eight criteria must be fulfilled, unless family history or molecular diagnosis is consistent with HLH. HLH-2004 chemo-immunotherapy includes etoposide, dexamethasone, cyclosporine A upfront and, in selected patients, intrathecal therapy with methotrexate and corticosteroids. Subsequent hematopoietic stem cell transplantation (HSCT) is recommended for patients with familial disease or molecular diagnosis, and patients with severe and persistent, or reactivated, disease. In order to hopefully further improve diagnosis, therapy and biological understanding, participation in HLH studies is encouraged.
M3 - SCORING: Zeitschriftenaufsatz
VL - 48
SP - 124
EP - 131
JO - PEDIATR BLOOD CANCER
JF - PEDIATR BLOOD CANCER
SN - 1545-5009
IS - 2
M1 - 2
ER -