Table 1.

Frequency of selection of the 28 MAS features by the 232 questionnaire respondents. median and mean ranks of features, and percentage of respondents attributing high rank to each feature.

FeatureNo. (%) Respondents Who Selected the FeatureMedian RankMean (SD) RankRespondents Giving Rank 8–10 to Feature, %Respondents Giving Rank 5–10 to Feature, %
Falling platelet count201 (86.6)6.56.1 (2.3)24.661.6
Hyperferritinemia194 (83.6)76.5 (3.0)39.253.9
Bone marrow hemophagocytosis188 (81.0)96.9 (3.6)44.855.2
Increased liver enzymes174 (75)55.0 (2.4)1340.9
Falling leukocyte count172 (74.1)55.6 (2.5)20.346
Persistent continuous fever ≥ 38°C158 (68.1)76.0 (3.4)30.240.1
Falling erythrocyte sedimentation rate142 (61.2)65.5 (2.7)15.938.8
Hypofibrinogenemia142 (61.2)55.4 (2.4)12.936.6
Hypertriglyceridemia135 (58.2)55.1 (2.7)16.831
Central nervous system dysfunction104 (44.8)55.0 (2.9)11.623.7
Falling hemoglobin level100 (43.1)54.8 (2.3)4.323.3
Prolongation of clothing times81 (34.9)4.54.5 (2.3)4.717.2
Increased D-dimer76 (32.8)55 (2.6)7.319.4
Hemorragic manifestations72 (31.0)55.3 (3.0)1017.7
Liver enlargement71 (30.6)44.8 (2.8)7.314.2
Spleen enlargement57 (24.6)44.5 (2.8)4.310.3
Increased lactic dehydrogenase45 (19.4)65.5 (2.6)4.711.6
Increased soluble IL-2 receptor α39 (16.8)65.1 (3.1)4.79.1
Increased soluble CD16327 (11.6)55.2 (3.0)4.36
Lymphadenopathy22 (9.5)44.4 (2.9)1.33.4
Decreased albumin19 (8.2)34.3 (2.9)1.32.6
Hyponatremia16 (6.9)55 (3.2)1.73.9
Arthritis improvement14 (6.0)23.1 (2.4)0.90.9
Renal failure13 (5.6)2.53.6 (2.9)0.91.7
Jaundice9 (3.9)55.9 (3.3)1.72.2
Increased bilirubin9 (3.9)23.4 (2.0)01.7
Respiratory failure6 (2.6)34.4 (3.0)0.40.9
Cardiac failure5 (2.2)33.8 (2.6)00.9