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HLH‐2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis
Author(s) -
Henter JanInge,
Horne AnnaCarin,
Aricó Maurizio,
Egeler R. Maarten,
Filipovich Alexandra H.,
Imashuku Shinsaku,
Ladisch Stephan,
McClain Ken,
Webb David,
Winiarski Jacek,
Janka Gritta
Publication year - 2007
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.21039
Subject(s) - hemophagocytic lymphohistiocytosis , medicine , hemophagocytosis , etoposide , hematopoietic stem cell transplantation , disease , pediatrics , immunology , chemotherapy , bone marrow , pancytopenia
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. Pediatr Blood Cancer 2007;48:124–131. © 2006 Wiley‐Liss, Inc.