Original ArticleDifferentiating Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis from Other Forms of Hemophagocytic Lymphohistiocytosis
Section snippets
Methods
Patient data were retrieved from the database of the German national HLH study center, to which patients are referred from Germany, Austria, and Switzerland. Patients with rheumatic disease are not routinely reported, but patients are registered if the center is contacted due to uncertainty about the diagnosis and/or treatment of MAS or if MAS is the presenting feature of a condition, such as sJIA. Diagnosis of MAS or HLH was made between 1992 and 2010. Data were obtained at diagnosis before
Characteristics of Patients with MAS and sJIA
The median age at the time of diagnosis of MAS in patients with MAS/sJIA was 10.0 years, and 59% were girls. In 22 of the 27 patients of the cohort, MAS was the first manifestation of sJIA. Arthritis was present initially in 17 and appeared only later in the course of disease in 5 of 22. In 5 patients, MAS manifested in previously diagnosed sJIA. The mean time between manifestation of sJIA and MAS in this subgroup was 5 years (range 0.75-10 years). Acute EBV infection was identified in 3
Discussion
MAS and FHL/VA-HLH share several features that not only comprise the substantial clinical overlap but include genetic and functional abnormalities, such as low perforin expression, perforin, and Munc13-4 sequence variants and reduced NK cytotoxicity.7, 14, 15, 16, 17 However, current treatment recommendations for MAS and FHL/VA-HLH differ substantially.1, 5, 10, 18, 19, 20, 21, 22 Thus, as the main focus of this study, measures were analyzed for their potential to discriminate between MAS in
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2022, Journal of Allergy and Clinical ImmunologyCitation Excerpt :The Dual-tg mice develop symptoms recapitulating an HLH-like disease, meanwhile showing HSPC compartment shifting toward enhanced myelopoiesis. Although lineage biases of the cytopenia presented in HLH are less commonly discussed, higher neutrophil numbers were reported in MAS than pHLH, with the former setting being more rationally represented by the Dual-tg model.59 In other entities of hyperinflammatory diseases, such as severe COVID-19, lineage biases (monocytosis, granulopoiesis, and increased neutrophil-to-lymphocyte ratios) are frequently noted and considered to play critical roles in driving disease pathogenesis.55,60
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2020, The Lancet RheumatologyDevelopment and Initial Validation of the Macrophage Activation Syndrome/Primary Hemophagocytic Lymphohistiocytosis Score, a Diagnostic Tool that Differentiates Primary Hemophagocytic Lymphohistiocytosis from Macrophage Activation Syndrome
2017, Journal of PediatricsCitation Excerpt :Aspartate aminotransferase and bilirubin did not discriminate well between the 2 conditions. Our results are similar to those reported by Lehmberg et al39 in a smaller sample. They found that higher neutrophil count and C-reactive protein and lower soluble CD25 indicated MAS rather than pHLH.
Similar but not the same: Differential diagnosis of HLH and sepsis
2017, Critical Reviews in Oncology/HematologyCitation Excerpt :In HLH it is associated with plasminogen activator released by macrophages (plasmin cleaves fibrinogen) (Janka, 2007a). In one study HLH was accompanied by coagulopathy in almost 95% (Palazzi et al., 2003) of pediatric patients, with a median fibrinogen concentration of 105 mg/dl; values from other studies ranged from 72 to 193 (Lehmberg et al., 2013; Niece et al., 2010; Ramachandran et al., 2011). In other pediatric (Janka and Schneider, 2004; Koh et al., 2015) and adult (Li et al., 2014b; Otrock and Eby, 2015; Parikh et al., 2014; Schram et al., 2016; Wang et al., 2015) groups hypofibrinogenemia was present in only 38–62%, which represents a low sensitivity; however higher percentages of 78–79% were described in other cohorts (Li et al., 2014a; Meeths et al., 2014).
The authors declare no conflicts of interest.
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Contributed equally.