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Table 2 Clinical features and laboratory data of patients with primary and visceral leishmaniosis-associated hemophagocytic lymphohistiocytosis

From: Comparison of the clinical features and outcome of children with hemophagocytic lymphohistiocytosis (HLH) secondary to visceral leishmaniasis and primary HLH: a single-center study

Variables   HLH classification P-Value
All patients (n = 60)
n (%)
Primary HLH (n = 40)
n (%)
VL-associated HLH (n = 20)
n (%)
Age
 <24 months 42 (70) 27 (67.5) 15 (75) 0.550
 ≥ 24 months 18 (30) 13 (32.5) 5 (25)
Sex
 Male 34 (56.7) 24 (60) 10 (50) 0.461
 Female 26 (43.3) 16 (40) 10 (50)
Fever
 <30 days 42 (70) 27 (67.5) 15 (75) >0.999
 ≥ 30 days 18 (30) 13 (32.5) 5 (25)
Hepatomegaly 36 (60) 24 (60) 12 (60) >0.999
Splenomegaly 58 (96.7) 39 (97.5) 19 (95) >0.999
Skin lesions 5 (8.3) 5 (12.5) 0 (0) 0.159
Bleeding tendency 5 (8.3) 5 (12.5) 0 (0) 0.159
CNS Involvement 23 (38.3) 18 (45) 5 (25) 0.133
Abnormal brain MRI 9 (15) 8 (20) 1 (5) 0.249
Bone marrow hemophagocytosis 6 (10) 6 (15) 0 (0) 0.165
Relapse 6 (10) 6 (15) 0 (0) 0.165
Death 26 (43.3) 26 (65) 0 (0) <  0.001*
H-score
 ≥ 169 56 (93.3) 37 (92.5) 19 (95) > 0.999
 < 169 4 (6.7) 3 (7.5) 1 (5)
ANC <1000/μl 51 (85) 34 (85) 17 (85) >0.999
Hemoglobin <9 g/dL 42 (70) 28 (70) 14 (70) >0.999
Platelet <100000/μl 40 (66.7) 31 (77.5) 9 (45) 0.012*
Bicytopenia 48 (80) 34 (85) 14 (70) 0.304
Triglyceride >265 mg/dL 41 (68.3) 28 (70) 13 (65) 0.695
Fibrinogen <150 mg/dL 32 (53.3) 20 (50) 12 (60) 0.464
Ferritin
 500-1000 ng/ml 3 (5) 3 (7.5) 0 (0.0) 0.147
 1000-10,000 ng/ml 41 (68.3) 29 (72.5) 12 (60)
 >10000 ng/ml 16 (26.7) 8 (20) 8 (40)
Ferritin, (ng/ml)
median (IQR)
4825
(2000.0-10,324.5)
3618
(1990.2-9479.2)
7122
(3226.2-33,112.2)
0.034*
D-dimer > 250 ng/ml 59 (98.3) 39 (97.5) 20 (100) >0.999
PT Prolonged
(age adjusted)
24 (40) 15 (37.5) 9 (45) 0.576
aPTT Prolonged
(age adjusted)
25 (41.7) 18 (45) 7 (35) 0.459
AST >2 × ULN (IU/L) 43 (71.7) 30 (75) 13 (65) 0.418
ALT >2 × ULN (IU/L) 25 (41.7) 21 (52.5) 4 (20) 0.016*
ESR > 20 mm/hr 31 (51.7) 16 (40) 15 (75) 0.011*
CRP ≥ 6 mg/L 53 (88.3) 33 (82.5) 20 (100) 0.084
**sCD25 ≥ 13.1 pg/mL 14 (70) 9 (64.3) 5 (83.3) 0.613
  1. VL visceral leishmaniosis, HLH hemophagocytic lymphohistiocytosis, ULN upper limit of normal, ANC absolute neutrophil count, PT prothrombin time, aPTT activated partial thromboplastin time, AST aspartate aminotransferase, ALT alanine aminotransferase, LDH lactic dehydrogenase, ESR Erythrocyte sedimentation rate, CRP C-reactive protein, CNS central nervous system, sCD25 soluble CD25
  2. *Statistically significant
  3. **sCD25 was measured in 20 patients