Macrophage Activation Syndrome (MAS) is a severe, life-threatening disorder. This disease was first described in association with systemic onset juvenile idiopathic arthritis (SJIA) [
The clinical and laboratory features of the MAS include high fever, hepatic dysfunction, encephalopathy, pancytopenia, bleeding disorders, and high ferritin [
Because of the similarities between MAS and hemophagocytic lymphohistiocytosis (HLH), some authors believed that the MAS was a secondary or acquired form of HLH [
Due to progressive and life-threatening course of the MAS, early diagnosis and treatment can be important in reducing morbidity and mortality. The criteria for the MAS diagnosis have some limitations in the early diagnosis. These criteria involves clinical and laboratory findings. The clinical criteria may appear in the later stages of disease [
The aims of the present study were comparing the laboratory data and changes in these data between the patients with MAS and the patients with flare-up of the autoimmune diseases and determine the cut-off points, sensitivity, and specificity of the static and dynamic laboratory data for the early diagnosis of MAS.
In a prospective study, 17 consecutive patients diagnosed with MAS, requiring hospital admission, between 2005 and 2014 that were admitted to the Children Medical Center entered the study. The patients over 18 years of age and the patients that did not return for follow-up were excluded from the study. Documented infection was not an exclusion criterion because of the triggering effect of the infection for MAS in the patients with rheumatologic diseases [
The criteria for diagnosis of the MAS in the patients with SJIA, polyarticular juvenile idiopathic arthritis (PJIA), and Kawasaki disease were obtained from the study of Ravelli and colleagues on the patients with SJIA [
The control group included 53 patients with the active disease of SJIA, PJIA, Kawasaki disease, and SLE that required hospital admission at the initial diagnosis (before starting treatment) or at the first flare-up. The control group patients were matched with the cases in age and sex. The criteria for active disease in JIA were based on the American College of Rheumatology (ACR) criteria in 2011 [
Age and gender of the patients were noted in the history taking. Bone marrow aspiration was done for all the patients in the MAS group and some of the patients in the control group to confirm the diagnosis.
The previous laboratory records were searched for white blood cell count (WBC), polymorphonuclear (PMN) and lymphocyte (Lymph) absolute count, hemoglobin (Hgb), platelet count (PLT), erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) in the initial phases of disease. These laboratory data were named as WBC1, PMN1, and so forth. The same laboratory data were obtained from the patients in the onset of MAS (MAS group) or active rheumatologic disease (control group) and named as WBC2, PMN2, and so forth. The same laboratory data were also obtained from the patients 1 month after discharge from the hospital and named as WBC3, PMN3, and so forth. The dynamic changes in these variables were assessed by subtracting the second set of laboratory data from the first and the third sets (e.g., WBC1-WBC2 and WBC3-WBC2). In the patients without previous records, only the second and third groups of data were used in the analysis.
In both groups, the following laboratory data were obtained at the time of first attack of MAS (MAS group) or first admission (control group): serum sodium, blood urea nitrogen (BUN), creatinine, lactate dehydrogenase (LDH), prothrombin time (PT), partial thromboplastin time (PTT), international normalized ratio (INR), total and direct bilirubin, alanine transaminase (ALT), aspartate aminotransferase (AST), alkaline phosphatase (ALP), and ferritin.
All the patients were treated according to the standard treatments for the underlying disease. The patients with MAS were also treated with methylprednisolone pulse (30 mg/kg/day, maximum 1 gram) for 3 days. The resistant or recurrent MAS were treated according to the guidelines of HLH in 2004 with dexamethasone, cyclosporine, and etoposide [
The number of the MAS attacks in the patients was also recorded. In the patients with more than one MAS attack, the data in the first attack were used in the analysis. The static laboratory data and dynamic changes in the selected data were compared between the MAS and control groups.
The statistical analysis was performed with SPSS version 20 (SPSS Inc., Chicago, IL). The Mann-Whitney
Seventy patients (17 cases in the MAS group) were included in the study. The characteristics of these patients were summarized in Table
The characteristics of patients. The quantitative variable (age) was shown as mean ± SD (range). The qualitative variables were shown as number (percentage).
MAS ( |
Control ( | |
---|---|---|
Age | ||
Mean ± SD (range) | 7.44 ± 4.4 (1–17) | 6.6 ± 3.93 (1–17) |
Gender | ||
Male | 10 (58.8%) | 38 (71.7%) |
Female | 7 (41.2%) | 15 (28.3%) |
Diagnosis | ||
SJIA | 10 (58.8%) | 31 (58.3%) |
PJIA | 4 (23.5%) | 12 (22.6%) |
Kawasaki | 2 (11.8%) | 6 (11.3%) |
SLE | 1 (5.9%) | 4 (7.5%) |
Presentation | ||
First presentation | 9 (52.9%) | 31 (58.5%) |
On background illness | 8 (47.1%) | 22 (41.5%) |
MAS: Macrophage Activation Syndrome, SD: standard deviation, SJIA: systemic onset juvenile idiopathic arthritis, PJIA: polyarticular juvenile idiopathic arthritis, and SLE: systemic lupus erythematosus.
In the control group, the admission of 31 patients was for the initial diagnosis and treatment. In the other 22 patients, the admission was due to flare-up of the underlying disease.
The mean, standard deviation, and range of the static laboratory data in the case and control groups and
The mean, standard deviation, and range of the laboratory data in the case and control groups and
MAS | Control (active disease) |
| |
---|---|---|---|
WBC1 (×103/uL) | 16122 ± 9105 (6100–38121) | 16280 ± 10857 (2520–61300) | 1 |
WBC2 (×103/uL) | 8784 ± 10310 (520–38430) | 15679 ± 8502 (700–41200) |
|
WBC3 (×103/uL) | 15617 ± 12602 (1130–46670) | 10299 ± 4551 (2140–25500) | 0.2 |
PMN1 (×103/uL) | 11664 ± 8500 (1449–34080) | 11174 ± 7870 (1128–36556) | 0.4 |
PMN2 (×103/uL) | 6775 ± 6569 (120–18069) | 11377 ± 7536 (583–35020) |
|
PMN3 (×103/uL) | 11472 ± 9539 (1142–31408) | 5834 ± 3462 (1001–17110) | 0.07 |
Lymph1 (×103/uL) | 4046 ± 4477 (866–19500) | 3815 ± 4441 (1108–32550) | 0.9 |
Lymph2 (×103/uL) | 2638 ± 3731 (639–15333) | 3635 ± 1812 (1209–8694) | < |
Lymph3 (×103/uL) | 3356 ± 2600 (550–8390) | 3501 ± 1917 (107–11272) | 0.4 |
Hgb1 (g/dL) | 9.13 ± 1.45 (6.80–12.20) | 10.40 ± 1.40 (7.50–14.3) |
|
Hgb2 (g/dL) | 7.96 ± 1.66 (6.10–11.4) | 10.33 ± 1.66 (7.30–14.5) | < |
Hgb3 (g/dL) | 8.95 ± 1.16 (6.90–10.70) | 11.58 ± 1.91 (6.50–15) | < |
PLT1 (/microliter) | 292588 ± 170526 (29000–638000) | 489365 ± 212249 (138000–1144000) |
|
PLT2 (/microliter) | 105882 ± 91791 (9000–406000) | 502617 ± 189080 (56000–1010000) | < |
PLT3 (/microliter) | 321785 ± 177361 (44000–770000) | 393512 ± 143221 (68000–779000) | 0.07 |
ESR1 (mm/h) | 69.53 ± 40.96 (9–126) | 73.67 ± 30.24 (5–125) | 0.7 |
ESR2 (mm/h) | 46.81 ± 49.47 (2–165) | 72.21 ± 35.82 (6–148) |
|
ESR3 (mm/h) | 37.53 ± 30.176 (10–102) | 23.78 ± 27.85 (3–140) | 0.1 |
CRP1 (mg/L) | 37 ± 23.61 (5–75) | 68 ± 52 (0–246) | 0.3 |
CRP2 (mg/L) | 139 ± 101 (40–345) | 96 ± 70 (22–317) | 0.1 |
CRP3 (mg/L) | 32.84 ± 46 (0.5–168) | 14.91 ± 19.4 (0–75) |
|
ALT (u/L) | 315 ± 840 (12–3560) | 28.28 ± 31.4 (6–187) | < |
AST (u/L) | 583 ± 1506 (17–6300) | 32.2 ± 21.7 (10–142) | < |
ALP (u/L) | 409 ± 290 (165–1220) | 392 ± 171 (84–941) | 0.3 |
ALB (gr/dL) | 3.03 ± 0.69 (2–4.3) | 3.80 ± 0.62 (2.7–4.7) |
|
Total Pr (gr/dL) | 6.42 ± 1.77 (4–8.7) | 6.90 ± 1.04 (4.9–8.4) | 0.5 |
BilT (mg/dL) | 5.36 ± 7.72 (0.4–24.3) | 0.88 ± 0.91 (0.4–2.7) |
|
BilD (mg/dL) | 3.32 ± 4.87 (0.1–14) | 0.26 ± 0.16 (0.2–0.6) | 0.2 |
Ferritin (ng/mL) | 60590 ± 73343 (1617–200000) | 5253 ± 5505 (300–18035) |
|
Fibrinogen (mg/dL) | 517 ± 641 (130–4944) | 489 ± 94 (423–556) | 0.2 |
LDH (Iu/L) | 1913 ± 1516 (136–4944) | 679 ± 336 (258–1318) |
|
BUN (mg/dL) | 16.75 ± 11.94 (5–41) | 10.18 ± 4.03 (4–26) | 0.1 |
Cr (mg/dL) | 0.60 ± 0.39 (0.12–1.80) | 0.55 ± 0.11 (0.3–0.8) | 0.5 |
Na (meq/L) | 130 ± 6.3 (115–137) | 136 ± 4.3 (128–144) |
|
TG (mg/dL) | 329 ± 188 (109–727) | 161 ± 119 (42–280) | 0.1 |
Chol (mg/dL) | 224 ± 130 (112–530) | 181 ± 131 (88–274) | 0.7 |
PT | 18.06 ± 7.7 (13–43) | 16.4 ± 7.9 (12–34) |
|
PTT | 43 ± 14 (18–75) | 31.50 ± 9.44 (13–45) |
|
INR | 1.67 ± 0.6 (1–2.7) | 1.16 ± 0.26 (1–1.8) |
|
MAS: Macrophage Activation Syndrome, WBC: white blood cells, PMN: polymorphonuclear, Lymph: lymphocytes, Hgb: hemoglobin, PLT: platelet, ESR: erythrocyte sedimentation rate, CRP: C-reactive protein, ALT: alanine aminotransferase, AST: aspartate aminotransferase, ALP: alkaline phosphatase, ALB: albumin, Total Pr: total protein, Bil T: total bilirubin, Bil D: direct bilirubin, LDH: lactate dehydrogenase, BUN: blood urea nitrogen, Cr: creatinine, Na: natrium, TG: triglycerides, Chol: cholesterol, PT: prothrombin time, PTT: partial prothrombin time, and INR: international normalized ratio. The previous laboratory records: WBC1, PMN1, and so forth. The laboratory data at the onset of MAS (MAS group) or active rheumatologic disease (control group): WBC2, PMN2, and so forth. The laboratory data 1 month after discharge from the hospital: WBC3, PMN3, and so forth.
The mean, standard deviation, and range of the dynamic changes in the laboratory data (e.g., WBC2-WBC3) in the case and control groups and
Differences between parameters detected at 2 different times in the case and control groups and
Parameter | MAS | Control |
|
---|---|---|---|
WBC1-WBC2 (×103/uL) | 7337 ± 11572 (−19030 to 34191) | 781.04 ± 9243 (−24000 to 43500) |
|
WBC3-WBC2 (×103/uL) | 8284 ± 14647 (−19640 to 44070) | −5593 ± 7941 (−28800 to 9830) |
|
PMN1-PMN2 (×103/uL) | 4888–10049 (−16228 to 31407) | −137 ± 6127 (−16908 to 12555) |
|
PMN3-PMN2 (×103/uL) | 6404 ± 9787 (−3750 to 29622) | −6167 ± 6288 (−21261 to 3572) | < |
Lymph1-Lymph2 (×103/uL) | 1277 ± 5889 (−11763 to 17670) | 381.78 ± 4864 (−6972 to 28278) | 0.07 |
Lymph3-Lymph2 (×103/uL) | 1484 ± 2505 (−1151 to 6560) | 135.45 ± 2504 (−6927 to 8025) | 0.1 |
Hgb1-Hgb2 (g/dL) | 1.24 ± 1.34 (−1.10 to 4.20) | 0.067 ± 1.38 (−2.50 to 2.90) |
|
Hgb3-Hgb2 (g/dL) | 1.17 ± 1.45 (−1.50 to 3.10) | 1.17 ± 1.75 (−2.40 to 5.10) | 0.8 |
PLT1-PLT2 (/microliter) | 190176 ± 176530 (11000–485000) | −2826 ± 176163 (−377000 to 353000) |
|
PLT3-PLT2 (/microliter) | 217785 ± 194694 (−2000 to 75800) | −90864 ± 164212 (−633000 to 140000) | < |
ESR1-ESR2 (mm/h) | 28 ± 35 (−40 to 84) | 2.65 ± 35.53 (−70 to 74) |
|
ESR3-ESR2 (mm/h) | 3.5 ± 60 (−91 to 155) | 49.68 ± 40.11 (−42 to 115) |
|
CRP1-CRP2 (mg/L) | 97.11 ± 93.75 (20 to 303) | 27.61 ± 83.73 (−174 to 252) |
|
CRP3-CRP2 (mg/L) | 116.95 ± 115.22 (0 to 299) | 80.20 ± 64.38 (21.60 to 269) | 0.5 |
MAS: Macrophage Activation Syndrome, WBC: white blood cells, PMN: polymorphonuclear, Lymph: lymphocytes, Hgb: hemoglobin, PLT: platelet, ESR: erythrocyte sedimentation rate, and CRP: C-reactive protein. The previous laboratory records: WBC1, PMN1, and so forth. The laboratory data at the onset of MAS (MAS group) or active rheumatologic disease (control group): WBC2, PMN2, and so forth. The laboratory data 1 month after discharge from the hospital: WBC3, PMN3, and so forth.
The results of the ROC curve analysis in static and dynamic variables were demonstrated in Tables
The results of receiver operating characteristic (ROC) curve analysis for static variables.
Parameter | ROC-AUC |
|
Cut-off value | Sensitivity | Specificity | 95% confidence interval |
---|---|---|---|---|---|---|
WBC1 (×103/uL) | 0.50 | 0.96 | — | — | — | 0.33–0.66 |
WBC2 (×103/uL) | 0.77 | 0.001 |
|
70% | 86% | 0.61–0.93 |
PMN1 (×103/uL) | 0.08 | 0.9 | — | — | — | 0.34–0.67 |
PMN2 (×103/uL) | 0.70 | 0.02 |
|
60% | 85% | 0.53–0.86 |
Lymph1 (×103/uL) | 0.50 | 0.9 | — | — | — | 0.33–0.68 |
Lymph2 (×103/uL) | 0.83 | <0.001 |
|
81% | 99.94% | 0.67–0.99 |
Hgb1 (g/dL) | 0.733 | 0.004 | ≤ |
58% | 83% | 0.58–0.87 |
Hgb2 (g/dL) | 0.83 | <0.001 | ≤ |
76% | 79% | 0.70–0.96 |
PLT1 (/microliter) | 0.76 | 0.001 | ≤ |
58% | 79% | 0.63–0.89 |
PLT2 (/microliter) | 0.96 | <0.001 | ≤ |
94% | 99.96% | 0.92–1.00 |
ESR1 (mm/h) | 0.41 | 0.3 | — | — | — | 0.22–0.60 |
ESR2 (mm/h) | 0.78 | 0.001 | ≤ |
81% | 72% | 0.65–0.92 |
CRP1 (mg/L) | 0.71 | 0.03 | ≤ |
61% | 69% | 0.54–0.85 |
CRP2 (mg/L) | 0.63 | 0.1 | ≥103 | 58% | 69% | 0.47–0.79 |
ALT (u/L) | 0.85 | <0.001 | ≥ |
82% | 84% | 0.73–0.97 |
AST (u/L) | 0.87 | <0.001 | ≥ |
82% | 78% | 0.76–0.98 |
ALP (U/L) | 0.42 | 0.4 | — | — | — | — |
ALB (gr/dl) | 0.86 | 0.001 | ≤ |
75% | 90% | 0.72–1.00 |
BilT (mg/dL) | 0.81 | 0.04 | ≥ |
66% | 84% | 0.59–1.00 |
BilD (mg/dL) | 0.69 | 0.2 | — | — | — | 0.42–0.96 |
Ferritin (ng/mL) | 0.81 | 0.01 | ≥ |
92% | 73% | 0.63–0.99 |
Fibrinogen (mg/dL) | 0.69 | 0.2 | — | — | — | 0.59–1.00 |
LDH (Iu/L) | 0.80 | 0.02 | ≥ |
72% | 80% | 0.63–1.00 |
BUN (mg/dL) | 0.63 | 0.1 | — | — | — | 0.46–0.81 |
Cr (mg/dL) | 0.44 | 0.6 | — | — | — | 0.25–0.64 |
Na (meq/L) | 0.81 | 0.005 | ≤ |
70% | 75% | 0.67–0.95 |
TG (mg/dL) | 0.79 | 0.1 | — | — | — | 0.49–1.00 |
Chol (mg/dL) | 0.61 | 0.6 | — | — | — | 0.064–1.00 |
PT | 0.74 | 0.04 | ≥ |
61.5% | 75% | 0.53–0.95 |
PTT | 0.79 | 0.01 | ≥ |
61 | 99 | 0.62–0.97 |
INR | 0.80 | 0.01 | ≥ |
84% | 67% | 0.62–0.97 |
ROC-AUC: receiver operating characteristic-area under the curve, MAS: Macrophage Activation Syndrome, WBC: white blood cells, PMN: polymorphonuclear, Lymph: lymphocytes, Hgb: hemoglobin, PLT: platelet, ESR: erythrocyte sedimentation rate, CRP: C-reactive protein, ALT: alanine aminotransferase, AST: aspartate aminotransferase, ALB: albumin, Total Pr: total protein, Bil T: total bilirubin, Bil D: direct bilirubin, LDH: lactate dehydrogenase, BUN: blood urea nitrogen, Cr: creatinine, Na: natrium, TG: triglycerides, Chol: cholesterol, PT: prothrombin time, PTT: partial prothrombin time, and INR: international normalized ratio. The previous laboratory records: WBC1, PMN1, and so forth. The laboratory data at the onset of MAS (MAS group) or active rheumatologic disease (control group): WBC2, PMN2, and so forth. The laboratory data 1 month after discharge from the hospital: WBC3, PMN3, and so forth.
The results of receiver operating characteristic (ROC) curve analysis for dynamic variables.
ROC-AUC |
|
Cut-off value | Sensitivity | Specificity | 95% confidence interval | |
---|---|---|---|---|---|---|
WBC1-WBC2 (×103/uL) | 0.73 | 0.004 |
|
70% | 77% | 0.59–0.88 |
WBC3-WBC2 (×103/uL) | 0.84 | <0.001 |
|
78% | 77% | 0.7–0.98 |
PMN1-PMN2 (×103/uL) | 0.69 | 0.02 |
|
73% | 70% | 0.53–0.86 |
PMN3-PMN2 (×103/uL) | 0.90 | <0.001 |
|
84% | 86% | 0.81–0.99 |
Lymph1-Lymph2 (×103/uL) | 0.65 | 0.09 | — | — | — | — |
Lymph3-Lymph2 (×103/uL) | 0.65 | 0.08 | — | — | — | — |
Hgb1-Hgb2 (g/dL) | 0.72 | 0.008 |
|
76% | 68% | 0.58–0.85 |
Hgb3-Hgb2 (g/dL) | 0.52 | 0.8 | — | — | — | — |
PLT1-PLT2 (/microliter) | 0.78 | 0.001 |
|
82% | 60% | 0.67–0.90 |
PL3-PLT2 (/microliter) | 0.92 | <0.001 |
|
78% | 90% | 0.85–1.00 |
ESR1-ESR2 (mm/h) | 0.69 | 0.02 |
|
64% | 56% | 0.54–0.85 |
ESR3-ESR2 (mm/h) | 0.80 | 0.002 | − |
75% | 86% | 0.63–0.97 |
CRP1-CRP2 (mg/L) | 0.77 | 0.003 |
|
76% | 67% | 0.64–0.89 |
CRP3-CRP2 (mg/L) | 0.53 | 0.5 | — | — | — | — |
ROC-AUC: receiver operating characteristic-area under the curve, MAS: Macrophage Activation Syndrome, WBC: white blood cells, PMN: polymorphonuclear, Lymph: lymphocytes, Hgb: hemoglobin, PLT: platelet, ESR: erythrocyte sedimentation rate, and CRP: C-reactive protein. The previous laboratory records: WBC1, PMN1, and so forth. The laboratory data at the onset of MAS (MAS group) or active rheumatologic disease (control group): WBC2, PMN2, and so forth. The laboratory data 1 month after discharge from the hospital: WBC3, PMN3, and so forth.
The differentiation between MAS and active diseases is one of the challenging problems in rheumatologic diseases, especially SJIA. These two conditions have overlapping clinical and laboratory features. So, the differentiation can be difficult, especially in early stages.
In this study, the static and dynamic features of the laboratory data were compared in two groups and according to the ROC curve analysis, best cut-off points, sensitivity, and specificity of each variable were determined. It was reported that, in active diseases, when WBC, PLT, and fibrinogen decreased from a high level to normal level, the diagnosis of MAS should be considered [
In this study, the highest value of AUC in the ROC curve analysis (Table
After PLT2, the most value of AUC belonged to the liver function tests (AST and ALT). The cut-off point for AST was 38.5 U/L and for ALT was 38 U/L. AST > 59 was a part of the MAS criteria [
The value of the AUC for albumin was as high as the value for AST and ALT. The calculated cut-off point for albumin in our study (3 mg/dL) was near the cut-off point in Ravelli and colleagues’ study (2.5 mg/dL) [
The AUC values were also significant for WBC2 and PMN2. In addition, these variables and their related dynamic variables (WBC2-WBC1, WBC3-WBC2, PMN1-PMN2, and PMN3-PMN2) were significantly different between case and control groups. The cut-off points for these variables (8200 × 103/UL for WBC2 and 3900 × 103/UL for PMN2) were higher than the cut-off points in other studies. These higher values might reflect that, in our patients, the diagnosis of MAS was done in an early stage with the changes in general condition, decrease in PLT, increase in liver enzymes and ferritin, and presence of hemophagocytes in the bone marrow. In this early stage, the WBC and PMN values were still in higher levels than later stages of disease. So, the higher cut-off points for WBC and PMN could be helpful for early diagnosis of MAS. In addition, the decrease in WBC > 3570 × 103/UL and the decrease in PMN > 1938 × 103/UL at the time of attack could also differentiate between MAS and active disease.
The lymphocyte count at the time of attack (Lymph2) had also high AUC value. This variable was also significantly lower in MAS group than control group. But the dynamic changes in this variable did not show significant difference between two groups. So, only lymphocyte counts at the time of MAS attack could help the diagnosis and the dynamic changes might not be helpful.
All three static Hgb variables and the dynamic variable Hgb1-Hgb2 were significantly different between two groups. The cut-off point for Hgb2 (8.9 g/dL) was similar to the cut-off point in the study of Kostik and colleagues (9 g/dL). So, the Hgb < 8.9 g/dL at the time of attack or decrease in Hgb > 0.55 g/dL from the previous records could help the diagnosis of MAS.
In contrast with other studies, the AUC for ferritin showed also high values. The cut-off point for ferritin was 5277 ng/mL. In the HLH criteria, the minimum threshold of ferritin for the diagnosis of HLH was 500 ng/mL [
In this study, the AUC for CRP2 was not significant. CRP1 and CRP2 showed no significant differences between case and control groups. In contrast, in previous studies, CRP had higher levels in MAS than in HLH [
The CRP value at the remission (CRP3) was also significantly higher in MAS group. The previous studies did not consider the laboratory data in the remission phase. CRP is a positive acute phase protein that is made in response to the cytokines released by macrophages [
The AUC for serum sodium showed high levels, with a cut-off point of 133 meq/L. This cut-off point was 130 meq/L in the Ravelli and colleagues’ study [
The main limitation of our study was low sample size. Higher sample size might yield more accurate results. Furthermore, the increase in the sCD163 and the decrease in C3 and C4, gene expression profiling (not evaluated in our study), were considered helpful in the differentiation of MAS from active disease in some studies. These laboratory exams may not be cost-effective and available in all centers. Further studies should be done to evaluate value of these laboratory exams in the diagnosis of MAS.
In conclusion, the dynamic changes in some laboratory data, especially PLT, can differentiate between MAS and active disease in the early phases with high sensitivity and specificity. The changes in WBC, PMN, and ESR and the levels of the liver enzymes may also be helpful in the early differentiation. Very high levels of ferritin may also help the diagnosis along with other clinical and laboratory signs.
Macrophage Activation Syndrome
White blood cells
Polymorphonuclear
Lymphocytes
Hemoglobin
Platelet
Erythrocyte sedimentation rate
C-reactive protein
Alanine aminotransferase
Aspartate aminotransferase
Lactate dehydrogenase
Prothrombin time
Partial prothrombin time
International normalized ratio
Receiver operating characteristic
The previous laboratory records
The laboratory data at the onset of MAS (MAS group) or active rheumatologic disease (control group)
The laboratory data 1 month after discharge from the hospital.
The authors declare that there is no conflict of interests regarding the publication of this study.