Comparison of clinical and laboratory features at disease onset between patients classified by the 28-member expert panel as having macrophage activation syndrome (MAS) (n=95) and those classified as not having MAS (n=296)
| Patients classified as having MAS | Patients classified as not having MAS | ||||
|---|---|---|---|---|---|
| No. with available data | No. (%) or median (IQR)* | No. with available data | No. (%) or median (IQR)* | p Value | |
| Clinical manifestations | |||||
| Fever | 94 | 93 (98.9) | 294 | 278 (94.6) | 0.08 |
| Hepatomegaly | 94 | 68 (72.3) | 295 | 75 (25.4) | <0.0001 |
| Splenomegaly | 92 | 53 (57.6) | 294 | 67 (22.8) | <0.0001 |
| Lymphadenopathy | 91 | 48 (52.8) | 292 | 73 (25.0) | <0.0001 |
| Central nervous system involvement | 93 | 40 (43.0) | 292 | 25 (8.6) | <0.0001 |
| Haemorrhagic manifestations | 92 | 25 (27.2) | 294 | 16 (5.4) | <0.0001 |
| Heart involvement | 94 | 27 (28.7) | 294 | 34 (11.6) | <0.0001 |
| Lung involvement | 95 | 27 (28.4) | 294 | 40 (13.6) | 0.0009 |
| Kidney involvement | 95 | 16 (16.8) | 295 | 16 (5.4) | 0.0004 |
| Laboratory results | |||||
| Haemoglobin, gm/dL | 95 | 9.9 (8.0–11.2) | 289 | 10.9 (9.4–12.2) | <0.0001 |
| Leucocyte count, ×109/L | 95 | 8.1 (3.2–12.8) | 289 | 15.3 (9.9–20.1) | <0.0001 |
| Neutrophil count, ×109/L | 82 | 3.7 (1.5–8.0) | 236 | 9.4 (5.1–14.2) | <0.0001 |
| Platelet count, ×109/L | 95 | 98 (57–141) | 290 | 385 (286–551) | <0.0001 |
| Erythrocyte sedimentation rate, mm/h | 90 | 28 (17–65) | 245 | 70 (39–93) | <0.0001 |
| C reactive protein, mg/dL | 85 | 8.7 (2.4–16.1) | 282 | 8.2 (2.4–15.6) | 0.63 |
| Aspartate aminotransferase, units/L | 93 | 171 (98–436) | 284 | 30 (22–45) | <0.0001 |
| Alanine aminotransferase, units/L | 91 | 115 (43–283) | 284 | 18 (12–34) | <0.0001 |
| Lactate dehydrogenase, units/L | 81 | 1560 (801–2400) | 248 | 482 (362–688) | <0.0001 |
| Triglycerides, mg/dL | 86 | 267 (192–358) | 186 | 123 (96–160) | <0.0001 |
| Albumin, gm/dL | 79 | 3.0 (2.6–3.5) | 252 | 3.7 (3.2–4.1) | <0.0001 |
| Serum sodium, mEq/L | 80 | 136 (133–140) | 259 | 138 (136–141) | 0.003 |
| Fibrinogen, mg/dL | 88 | 220 (148–345) | 226 | 500 (356–650) | <0.0001 |
| Ferritin, ng/mL | 90 | 9094 (2000–19 767) | 244 | 268 (62–938) | <0.0001 |
| D-dimer, ng/mL | 48 | 3579 (1834–7373) | 94 | 1638 (528–3325) | <0.0001 |
*Clinical manifestations are reported as the number (%) and laboratory results as the median (IQR).