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