Development and Validation of the HScore, a Score for the Diagnosis of Reactive Hemophagocytic Syndrome

Abstract

Objective. Because it has no unique clinical, biologic, or histologic features, reactive hemophagocytic syndrome may be difficult to distinguish from other diseases such as severe sepsis or hematologic malignancies. This study was undertaken to develop and validate a diagnostic score for reactive hemophagocytic syndrome.Methods. A multicenter retrospective cohort of 312 patients who were judged by experts to have reactive hemophagocytic syndrome (n ‫؍‬ 162), were judged by experts to not have reactive hemophagocytic syndrome (n ‫؍‬ 104), or in whom the diagnosis of reactive hemophagocytic syndrome was undetermined (n ‫؍‬ 46) was used to construct and validate the reactive hemophagocytic syndrome diagnostic score, called the HScore. Ten explanatory variables were evaluated for their association with the diagnosis of hemophagocytic syndrome, and logistic regression was used to calculate the weight of each criterion included in the score. Performance of the score was assessed using developmental and validation data sets.

Macrophage activation syndrome in adult systemic lupus erythematosus: report of seven adult cases from a single Italian rheumatology center

“…Here we present the largest population of adult SLE-associated MAS evaluated by the H-score so far. Our data seem to confirm the utility of the H-score in this setting, even if it should always be considered that sensitivity may not be 100% (13,17). However, since in our study all the patients also fulfilled the HLH-2004 classification criteria (10), it was not possible to evaluate the sensitivity of the H-score as compared with the HLH-2004 criterion, the main limitation of which is indeed the lack of sensitivity, especially at the beginning of the MAS.…”

“…Median HLH score was 248 (IQR 226-263), with a median probability of having MAS of 99% (Table II). In all patients the Hscore was higher than 169, the cut-off for classification of reactive MAS in the original study (13); however, in one patient the score was lower than 190.5, a cut-off suggested by other authors to better perform in the classification of reactive MAS in patients with rheumatic diseases (17). In three cases bone marrow aspirate was not performed; nevertheless, the H-score was higher than the suggested cut-offs in all these cases (Table II).…”

“…Recently, preliminary diagnostic guidelines for MAS complicating systemic juvenile idiopathic arthritis (11), or juvenile SLE (12), have been published, but these may not be suitable for adult patients. Finally, a diagnostic score (H-score) was developed with the aim of estimating the individual risk of having MAS for adult patients (13). However, the majority of patients in this study population had haematological malignancy and/or infection, and only 3.5% of them had SLE.…”

Weathering a Macrophage Storm

“…Using this strict set of criteria may delay diagnosis in patients with a less severe initial presentation, putting them at risk for rapid clinical decline and potential death from delayed treatment. To improve upon this, Fardet, et al developed a modified scoring system (HScore) 12 , based on the HLH-2004 criteria, to estimate a patient's risk of having secondary HLH; however, this has not been validated in patients with an underlying rheumatic systemic inflammatory condition such as sJIA or AOSD. In fact, a majority of patients included in this study had HLH secondary to an underlying malignancy or infection 12 .…”

An unusual extranodal T-cell non-Hodgkin lymphoma

“…In addition, the absence of haemophagocytosis made the case for HLH less compelling (probability HScore 70%). 2 With a provisional diagnosis of 'idiopathic inflammatory myopathy', we decided to immunosuppress her with intravenous methylprednisolone. On this occasion, the marrow was unaspirable and the trephine biopsy showed neartotal effacement of the marrow by a necrotic, high-grade tumour (figure B).…”