It may be tough to distinguish hemophagocytic syndrome from sepsis or hematologic malignancies as it does not have any biological, clinical, or histologic features. The objective of this study was to develop a diagnostic score for reactive hemophagocytic syndrome and establish its validation. As a part of this study, 312 patients were evaluated by the experts to check symptoms of reactive hemophagocytic syndrome. Out of which, 162 patients were affected by reactive hemophagocytic syndrome while 104 patients did not show symptoms of this disease and the 46 cases were undetermined.
The data was used to develop the reactive hemophagocytic syndrome and the validation of diagnostic score known as HScore. Besides, the evaluation of the association with the diagnosis of the hemophagocytic syndrome was assessed by the ten variables along with the logistic regression. The process was aimed at the calculation of HScore further understanding the performance of the score through developmental and validation data sets.
From the study, nine variables, namely, 5 biological, 3 clinical, and 1 cytologic feature of bone marrow were recorded in the HScore. 0-18 was the average number of points assigned to all the variables while 0-64 points were assigned to immunosuppression for triglyceride level. 230 was the median HScore that resulted from the assessment indicating the positive diagnosis of the reactive hemophagocytic syndrome and 125 for the negative diagnosis.
Range from <1% with an HScore of ≤90 to >99% with an HScore of ≥250 was the probability to diagnose the hemophagocytic syndrome.