Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long-term results of the cooperative HLH-2004 study

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Confirmed efficacy of etoposide and dexamethasone in HLH treatment : long-term results of the cooperative HLH-2004 study

Bergsten, Elisabet (författare): Karolinska Institutet

Horne, AnnaCarin (författare): Karolinska Institutet

Aricó, Maurizio (författare): Direzione Generale, Azienda Sanitaria Provinciale, Ragusa, Italy

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Astigarraga, Itziar (författare): Servicio de Pediatria, BioCruces Health Research Institute, Hospital Universitario Cruces, Universidad del Pais Vasco–Euskal Herriko Unibertsitatea (UPV/EHU), Barakaldo, Spain

Egeler, R. Maarten (författare): Division of Hematology & Oncology, The Hospital for Sick Children, Toronto ON, Canada

Filipovich, Alexandra H. (författare): Division of Bone Marrow Transplant and Immune Deficiency, Cincinnati Children’s Hospital Medical Center, Cincinnati OH, USA

Ishii, Eiichi (författare): Department of Pediatrics, Graduate School of Medicine, Ehime University, Ehime, Japan

Janka, Gritta (författare): Pediatric Hematology and Oncology, University Medical Center Hamburg, Hamburg, Germany

Ladisch, Stephan (författare): Center for Cancer and Immunology Research, Children’s Research Institute, Children’s National Medical Center, Washington DC, USA

Lehmberg, Kai (författare): Pediatric Hematology and Oncology, University Medical Center Hamburg, Hamburg, Germany

McClain, Kenneth L. (författare): Texas Children’s Cancer Center, Baylor College of Medicine, Houston TX, USA

Minkov, Milen (författare): University Clinic of Pediatrics, St. Anna Children’s Hospital, Medical University of Vienna, Vienna, Austria

Montgomery, Scott, 1961- (författare): Karolinska Institutet,Örebro universitet,Institutionen för medicinska vetenskaper,Clinical Epidemiology Unit, Department of Medicine, Solna, Karolinska Institutet, Stockholm, Sweden; Department of Epidemiology and Public Health, University College London, London, United Kingdom,Clinical Epidemiology and Biostatistics

Nanduri, Vasanta (författare): Department of Pediatrics, Watford General Hospital, Watford, United Kingdom

Rosso, Diego (författare): Department of Pediatric Hematology and Oncology, Hospital de Niños Dr Pedro De Elizalde, Buenos Aires, Argentina; Department of Pediatrics, Hospital de Clinicas Jose de San Martin, Buenos Aires, Argentina; Consejo Nacional de Investigaciones Científicas y Técnicas (CONICET), Buenos Aires, Argentina

Henter, Jan-Inge (författare): Karolinska Institutet

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(creator_code:org_t)

American Society of Hematology, 2017
2017
Engelska.
Ingår i: Blood. - : American Society of Hematology. - 0006-4971 .- 1528-0020. ; 130:25, s. 2728-2738

Relaterad länk:: https://ashpublicati...; visa fler...; https://urn.kb.se/re...; https://doi.org/10.1...; http://kipublication...; visa färre...

Tidskriftsartikel (refereegranskat)

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Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome comprising familial/genetic HLH (FHL) and secondary HLH. In the HLH-94 study, with an estimated 5-year probability of survival (pSu) of 54% (95% confidence interval, 48%-60%), systemic therapy included etoposide, dexamethasone, and, from week 9, cyclosporine A (CSA). Hematopoietic stem cell transplantation (HSCT) was indicated in patients with familial/genetic, relapsing, or severe/persistent disease. In HLH-2004, CSA was instead administered upfront, aiming to reduce pre-HSCT mortality and morbidity. From 2004 to 2011, 369 children aged <18 years fulfilled HLH-2004 inclusion criteria (5 of 8 diagnostic criteria, affected siblings, and/or molecular diagnosis in FHL-causative genes). At median follow-up of 5.2 years, 230 of 369 patients (62%) were alive (5-year pSu, 61%; 56%-67%). Five-year pSu in children with (n = 168) and without (n = 201) family history/genetically verified FHL was 59% (52%-67%) and 64% (57%-71%), respectively (familial occurrence [n = 47], 58% [45%-75%]). Comparing with historical data (HLH-94), using HLH-94 inclusion criteria, pre-HSCT mortality was nonsignificantly reduced from 27% to 19% (P = .064 adjusted for age and sex). Time from start of therapy to HSCT was shorter compared with HLH-94 (P =020 adjusted for age and sex) and reported neurological alterations at HSCT were 22% in HLH-94 and 17% in HLH-2004 (using HLH-94 inclusion criteria). Five-year pSu post-HSCT overall was 66% (verified FHL, 70% [63%-78%]). Additional analyses provided specific suggestions on potential pre-HSCT treatment improvements. HLH-2004 confirms that a majority of patients may be rescued by the etoposide/dexamethasone combination but intensification with CSA upfront, adding corticosteroids to intrathecal therapy, and reduced time to HSCT did not improve outcome significantly.

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Av författaren/redakt...: Bergsten, Elisab ...; Horne, AnnaCarin; Aricó, Maurizio; Astigarraga, Itz ...; Egeler, R. Maart ...; Filipovich, Alex ...; visa fler...; Ishii, Eiichi; Janka, Gritta; Ladisch, Stephan; Lehmberg, Kai; McClain, Kenneth ...; Minkov, Milen; Montgomery, Scot ...; Nanduri, Vasanta; Rosso, Diego; Henter, Jan-Inge; visa färre...

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MEDICIN OCH HÄLSOVETENSKAP: MEDICIN OCH HÄLS ...; och Klinisk medicin; och Hematologi

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Av lärosätet: Örebro universitet; Karolinska Institutet

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Confirmed efficacy of etoposide and dexamethasone in HLH treatment : long-term results of the cooperative HLH-2004 study

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