HLH‐2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis
Jan‐Inge Henter, AnnaCarin Horne, Maurizio Aricò et al. · 25 Aug 2006
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…
Langerhans cell histiocytosis (LCH): Guidelines for diagnosis, clinical work‐up, and treatment for patients till the age of 18 years
Riccardo Haupt, Milen Minkov, Itziar Astigarraga et al. · 25 Oct 2012
Abstract These guidelines for the management of patients up to 18 years with Langerhans cell histiocytosis (LCH) have been set up by a group of experts involved in the Euro Histio Net project who participated in national or international studies and in peer reviewed publications. Existing guidelines were reviewed and changed where new evidence was…
Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis
Carl E. Allen, Xiaoying Yu, Claudia A. Kozinetz et al. · 17 Dec 2007
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a potentially lethal condition characterized by a pathologic inflammation. The diagnostic criteria for HLH include fever, splenomegaly, cytopenias, hypertriglyceridemia, hypofibrinogenemia, abnormal natural killer cell (NK cell) functional assay, elevated soluble IL-2Ralpha level, and elevated ferritin level (>500 microg/L). Institution of timely therapy in these critically ill patients may be delayed…
Sirolimus for the treatment of complicated vascular anomalies in children
Adrienne M. Hammill, MarySue Wentzel, Anita Gupta et al. · 28 Mar 2011
BACKGROUND: Vascular anomalies comprise a diverse group of diagnoses. While infantile hemangiomas are common, the majority of these conditions are quite rare and have not been widely studied. Some of these lesions, though benign, can impair vital structures, be deforming, or even become life-threatening. Vascular tumors such as kaposiform hemangioendotheliomas (KHE) and complicated vascular malformations…
The pediatric preclinical testing program: Description of models and early testing results
Peter J. Houghton, Christopher L. Morton, Chandra L. Tucker et al. · 25 Oct 2006
BACKGROUND: The Pediatric Preclinical Testing Program (PPTP) is an initiative supported by the National Cancer Institute (NCI) to identify novel therapeutic agents that may have significant activity against childhood cancers. The PPTP has established panels of childhood cancer xenografts and cell lines to be used for in vivo and in vitro testing. These include panels…
