Table 4. Comparison of characteristics between survivors and non-survivors in patients with MAS.
| Survivors (n = 13) | Non-survivors (n = 7) | p-value | |
|---|---|---|---|
| Sex, female, n (%) | 12 (92.3) | 6 (85.7) | 1.000 |
| Age, years, mean ± SD | 34.6 ± 14.5 | 44.4 ± 19.4 | 0.215 |
| Disease duration to MAS occurrence, months, median (IQR) | 1.0 (0.0, 96.0) | 0 (0.0, 1.0) | 0.393 |
| Clinical features | |||
| Splenomegaly, n (%) | 10 (76.9) | 3 (42.9) | 0.174 |
| Hepatomegaly, n (%) | 6 (46.2) | 1 (14.3) | 0.329 |
| Lymphadenopathy, n (%) | 10 (76.9) | 4 (57.1) | 0.613 |
| Pneumonitis, n (%) | 3 (23.1) | 3 (42.9) | 0.613 |
| Renal impairment¶¶, n (%) | 1 (7.7) | 5 (71.4) | 0.007 |
| Neuropsychiatric manifestations, n (%) | 5 (38.5) | 1 (14.3) | 0.354 |
| Lab findings | |||
| ANC, /uL, median (IQR) | 2880 (685, 6475) | 3240 (890, 9260) | 0.588 |
| Hb, g/dL, median (IQR) | 8.7 (7.6, 9.2) | 7.6 (6.4, 8.9) | 0.183 |
| PLT, × 109/L, median (IQR) | 90.0 (61.0, 149.0) | 43.0 (30.0, 74.0) | 0.002 |
| AST, IU/L, median (IQR) | 364.0 (137.0, 689.0) | 81.0 (37.0, 157.0) | 0.019 |
| LDH, IU/L, median (IQR) | 1346.0 (655.5, 1893.5) | 343.0 (279.0, 733.0) | 0.014 |
| TB, mg/dL, median (IQR) | 0.8 (0.4, 1.8) | 0.8 (0.5, 4.5) | 0.699 |
| Albumin, g/dL, median (IQR) | 2.4 (2.1, 2.7) | 2.2 (1.8, 2.8) | 0.588 |
| ESR, mm/hr, median (IQR) | 28.0 (17.5, 52.0) | 23.0 (6.0, 67.0) | 0.588 |
| CRP, mg/dL, median (IQR) | 1.6 (0.4, 4.0) | 6.1 (2.2, 22.0) | 0.056 |
| Ferritin, ng/mL, median (IQR) | 16817.0 (2811.5, 48286.0) | 5409.7 (2784.1, 6395.3) | 0.183 |
| TG, mg/dL, median (IQR) | 322.5 (209.5, 436.3) | 176.0 (137.0, 403.0) | 0.299 |
| Fibrinogen, mg/dL, median (IQR) | 214.0 (148.5, 261.0) | 217.0 (176.0, 363.0) | 1.000 |
| Low/absent NK-cell activity | 5 (38.5) | 1 (14.3) | 0.354 |
| Soluble CD25 ≥ 2400 U/ml | 3 (23.1) | 5 (71.4) | 0.062 |
| Hemophagocytosis, n (%) | 11 (84.6) | 5 (71.4) | 0.587 |
| Hscore*, mean ± SD | 251.9 ± 37.8 | 220.9 ± 47.5 | 0.135 |
| Combined infection | 5 (38.5) | 5 (71.4) | 0.350 |
| Previous long-term steroid use, n (%) | 7 (53.8%) | 1 (14.3%) | 0.158 |
| Rheumatic disease | 0.354 | ||
| SLE | 8 (61.5) | 6 (85.7) | |
| AOSD | 5 (38.5) | 1 (14.3) |
*HScore calculator (for the percentage probability of secondary HLH) is available at http://saintantoine.aphp.fr/score/.
¶¶Renal impairment was defined as an abrupt (within 48 hours) >50% decrease in eGFR or the need for renal replacement therapy (dialysis) during MAS treatment. MAS: Macrophage activation syndrome, HLH: Hemophagocytic lymphohistiocytosis, ANC: Absolute neutrophil count, Hb: Hemoglobin, PLT: Platelet, AST: Aspartate aminotransferase, LDH: Lactate dehydrogenase, TB: Total bilirubin, ESR: Estimated sedimentation rate, CRP: C-reactive protein, TG: Triglyceride, SLE: Systemic lupus erythematosus, AOSD: Adult-onset still’s disease, eGFR, estimated glomerular filtration rate