Table 2.
Clinical features and laboratory data of patients with primary and visceral leishmaniosis-associated hemophagocytic lymphohistiocytosis
| 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 |
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
*Statistically significant
**sCD25 was measured in 20 patients