Hemophagocytic syndromes (HPS) including Hemophagocytic Lymphohistiocytosis (HLH) in Adults: A Systematic Scoping Review

• Published in: Blood reviews Vol. 30; no. 6; pp. 411 - 420
• Main Authors: Hayden, Anna, Park, Sujin, Giustini, Dean, Lee, Agnes Y.Y, Chen, Luke Y.C
• Format: Journal Article
• Language: English
• Published: England Elsevier Ltd 01-11-2016
• Subjects: Adults; Combined Modality Therapy; Disease Susceptibility; Hematology, Oncology and Palliative Medicine; Hematophagic histiocytosis; Hemophagocytic lymphohistiocytosis; Hemophagocytic syndromes; Humans; Hyperferritinemia; Lymphohistiocytosis, Hemophagocytic - diagnosis; Lymphohistiocytosis, Hemophagocytic - epidemiology; Lymphohistiocytosis, Hemophagocytic - etiology; Lymphohistiocytosis, Hemophagocytic - therapy; Macrophage activation syndromes; Practice Guidelines as Topic; Prognosis; Treatment Outcome; Hemophagocytic lymphohistiocytosis; macrophage activation syndromes; hemophagocytic syndromes; hematophagic histiocytosis; adults; hyperferritinemia; Hemophagocytic syndromes; Hyperferritinemia; Hematophagic histiocytosis; Adults; Macrophage activation syndromes
• ISSN: 0268-960X; 1532-1681
• DOI: 10.1016/j.blre.2016.05.001

Abstract Most knowledge of hemophagocytic syndromes (HPS) 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 > 15 years) 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.