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Table 5 Demographic characteristics and laboratory variables of patients with secondary HLH/MAS treated with PE and methylprednisolone or IVIG or with PE and chemotherapy or dexamethasone

From: Hyperferritinemia in the critically ill child with secondary hemophagocytic lymphohistiocytosis/sepsis/multiple organ dysfunction syndrome/macrophage activation syndrome: what is the treatment?

  PE and methylprednisolone or IVIG (n= 17) PE and chemotherapy or dexamethasone (n= 6)  
  Frequency, percentage Number   Frequency, percentage Number   P
Males/Females 88/12 15/2   33/67 2/4   0.003
Underlying disease 77 13   83 5   0.6
Mechanical ventilation 89 15   83 5   0.5
  Mean ± SD Median Range Mean ± SD Median Range  
Age, years 6.8 ± 3.3 6.5 2-14 7.6 ± 4.8 6.8 2.8-15.1 0.8
PRISM III-24 score 25.6 ± 13.4 25.6 7-48 19.5 ± 8.8 17.5 11-53 0.3
PELOD score 26.2 ± 13.8 24 4-52 27.5 ± 15. 26.5 13-53 0.9
Number of organs involved 4.5 ± 1 5 3-6 4.6 ± 1 5 3-6 0.7
Hemoglobin, g/dL 7.3 ± 1.5 8 4.8-8.9 8.8 ± 3.3 8.3 5.2-15 0.4
Leukocytes,/mm3 10,145 ± 9,230 6,270 500-26,400 12,096 ± 17189 6,500 400-46,600 0.9
Absolute neutrophil count,/mm3 7,420 ± 7,598 7,420 100-23,300 8,801 ± 13,291 4,475 0-35,000 0.6
Platelet,/mm3 51,594 ± 42,767 45,000 10,000-187,000 80,833 ± 81,052 70,000 12,000-235,000 0.6
PT, seconds 29.2 ± 14.68 25 13-69 14 ± 2.1 14.1 10.3-16.5 0.004
aPTT, seconds 45.2 ± 13.1 45.8 30-74 33.2 ± 14 25.7 24.7-59.3 0.05
Fibrinogen, mg/dL 188.3 ± 169 148.5 0-607 206 ± 139 206 110-443 0.2
Triglycerides, mg/dL 515 ± 275 450 122-1,094 654 ± 507 547 108-1,511 0.9
Albumin, mg/dL 2.6 ± 0.7 2.7 1.4-4.1 2.7 ± 0.3 2.6 2.4-3.3 0.9
Sodium, mEq/L 131 ± 6.9 131 120-141 136 ± 6.7 135 130-147 0.1
LDH, U/L 42,10 ± 3306 2,300 750-11,270 2,349 ± 1,934 2,310 410-4,374 0.4
ALT, U/L 310 ± 627 100 21-2,362 3,080 ± 5,466 409 227-11,276 0.03
Ferritin, μg/dL 20,538 ± 28,199 5,109 765-100,000 38,844 ± 38,130 31,718 2,998-97,767 0.2
MV duration, days 18.7 ± 17.4 12 2-65 20 ± 25 5 1-60 0.6
PICU LOS, days 23 ± 18.8 16 4-69 21.6 ± 23 12.9 1-60 0.5
Hospital LOS, days 43.9 ± 33.7 30 11-110 39.2 ± 26.3 41.5 5-70 0.8
  Frequency Number   Frequency Number   
Survival 100% 17   50% 3   0.002
  1. ALT, alanine aminotransferase; aPTT, activated partial thromboplastin time; HLH, hemophagocytic lymphohistiocytosis; IVIG, intravenous immunoglobulin; LDH, lactate dehydrogenase; LOS, length of stay; MAS, macrophage activation syndrome; MV, mechanical ventilation; PE, plasma exchange; PELOD, Pediatric Logistic Organ Dysfunction; PICU, pediatric intensive care unit; PRISM, Pediatric Risk of Mortality; PT, Prothrombin time; SD, standard deviation.