title: Hemophagocytic syndromes (HPSs) including hemophagocytic lymphohistiocytosis (HLH) in adults: A systematic scoping review.
abstract: Most knowledge of hemophagocytic syndromes (HPSs) including hemophagocytic lymphohistiocytosis (HLH) is derived from pediatric studies; literature on adult HPS/HLH predominantly consists of small retrospective studies with clinical and methodological heterogeneity. The aims of this systematic scoping review were to provide an overview of existing literature on adult HPS/HLH, describe current practices in diagnosis and treatment, and propose priorities for future research. Articles from Ovid Medline, Embase and Pubmed (1975-2015) describing 10 or more unique adults (age>15years) with HPS/HLH were included. 82 publications were eligible: 10 were prospective and 72 were retrospective. Of the six distinct diagnostic criteria, the HLH-2004 criteria were by far the most commonly used. A minority of studies tested for genetic abnormalities (12), soluble interleukin-2 receptor (11), and/or NK function (11) in a subset of patients. Most centers used steroids and either etoposide-based (HLH-94/HLH-2004) or doxorubicin-based (CHOP) initial therapy regimens. Allogeneic hematopoietic cell therapy for treatment of adult HLH has rarely been reported. Mortality in larger treatment focused studies ranged from 20 to 88%. Developing adult-specific diagnostic criteria based on widely evaluable features of secondary HPS/HLH and establishing standard initial therapies are priorities for future research.
authors.affiliation: Division of Hematology, Department of Medicine, University of British Columbia, Vancouver, Canada.
authors.forename: Anna
authors.initials: A
authors.lastname: Hayden
authors.affiliation: Division of Hematology, Department of Medicine, University of British Columbia, Vancouver, Canada.
authors.forename: Sujin
authors.initials: S
authors.lastname: Park
authors.affiliation: Biomedical Branch Library, University of British Columbia, Vancouver, Canada.
authors.forename: Dean
authors.initials: D
authors.lastname: Giustini
authors.affiliation: Division of Hematology, Department of Medicine, University of British Columbia, Vancouver, Canada.
authors.forename: Agnes Y Y
authors.initials: AY
authors.lastname: Lee
authors.affiliation: Division of Hematology, Department of Medicine, University of British Columbia, Vancouver, Canada. Electronic address: lchen2@bccancer.bc.ca.
authors.forename: Luke Y C
authors.initials: LY
authors.lastname: Chen
mesh_terms: D003131:Combined Modality Therapy; D004198:Disease Susceptibility; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology / Q000628:therapy*; D017410:Practice Guidelines as Topic; D011379:Prognosis; D016896:Treatment Outcome
keywords: Adults; Hematophagic histiocytosis; Hemophagocytic lymphohistiocytosis; Hemophagocytic syndromes; Hyperferritinemia; Macrophage activation syndromes