Table 2.
Clinical and Labs Characteristics of Adult Patients with HLH According to Etiologies
| Variables | Overall (n = 52) | Female (n = 23) | Male (n = 29) | p-value | M-HLH (n = 22) | I-HLH (n = 20) | MAS-HLH (N = 7) | Idiopatic-HLH (n = 2)* |
|---|---|---|---|---|---|---|---|---|
| Total Effective (%) or Mean (SD) | Total Effective (%) or Mean (SD) | Total Effective (%) or Mean (SD) | Total Effective (%) or Mean (SD) | Total Effective (%) or Mean (SD) | Total Effective (%) or Mean (SD) | Total Effective (%) or Mean (SD) | ||
| Gender (male) | 29 (56%) | / | / | / | 14 (64%) | 13 (65%) | 0 | 2 (100%) |
| Mean age (SD) | 48 (18) | 45 (17) | 50 (20) | 0.27 | 56 (21) | 42 (15) | 39 (11) | 43 (22) |
| Fever | 51 (98%) | 23 (100%) | 28 (97%) | 0.37 | 22 (100%) | 19 (95%) | 7 (100%) | 2 (100%) |
| Splenomegaly | 35 (67%) | 14 (61%) | 21 (72%) | 0.38 | 16 (73%) | 14 (70%) | 3 (43%) | 1 (50%) |
| Hepatomegaly | 14 (27%) | 8 (35%) | 6 (21%) | 0.25 | 8 (36%) | 3 (15%) | 2 (29%) | 0 |
| Bicytopenia | 48 (92%) | 23 (100%) | 25 (86%) | 0.064 | 22 (100%) | 17 (85%) | 7 (100%) | 1 (50%) |
| ANC (mm³/µL) | 4297 (4938) | 4913 (5692) | 3421 (3622) | 0.28 | 4206 (4263) | 4790 (5897) | 4250 (5170) | 460 (424) |
| Hb (g/dl) | 9.2 (1.9) | 8.6 (1.3) | 9.6 (2.2) | 0.051 | 8.7 (1.6) | 9.9 (2.3) | 9.1 (1.4) | 7.9 (1.5) |
| Pt (mm³/µL) | 69,784 (57,862) | 66,695 (50,416) | 72,551 (63,000) | 0.71 | 62,142 (58,454) | 70,950 (60,698) | 81,428 (54,859) | 55,000 (36,062) |
| TG (mg/dl) | 323(128) | 348 (138) | 293 (111) | 0.13 | 302 (119) | 342 (115) | 267 (130) | 487 (269) |
| Fib (mg/dl) | 358 (264) | 320 (284) | 387 (230) | 0.36 | 434 (303) | 311 (252) | 277 (164) | 287 (251) |
| Ferritin µg/L | 32315 (145,382) | 13,346 (192,780) | 46,345 (11,837) | 0.42 | 59,630 (226,275) | 13,587 (16,173) | 14,978 (12,835) | 4348 (1199) |
| ALAT (U/L) | 285 (643) | 197 (181) | 355 (846) | 0.39 | 99 (120) | 530 (985) | 199 (193) | 101 (7.8) |
| ASAT (U/L) | 235 (119) | 246 (274) | 355 (846) | 0.84 | 198 (274) | 356 (495) | 259 (336) | 120 (45) |
| TBIL (mg/dl) | 3.2 (5.8) | 4.6 (7.4) | 2.1 (3.6) | 0.11 | 2.6 (6.3) | 2.6 (3.2) | 2.1 (2.9) | 9.6 (11.9) |
| Albumin (mg/dl) | 28 (5.9) | 28 (6.4)(6.4) | 29 (5.3) | 0.3 | 29 (5.2) | 27 (6.7) | 27 (5.8) | 31 (7.7) |
| LDH (UI/L) | 1115 (1078) | 956 (838) | 1241 (1235) | 0.35 | 1055 (972) | 1238 (1226) | 1085 (1207) | 314 (69) |
| Cortitosteroids | 42 (81%) | 20 (87%) | 22 (76%) | 0.31 | 20 (91%) | 12 (60%) | 7 (100%) | 2 (100%) |
| Etoposide | 8 (15%) | 4 (17%) | 4 (14%) | 0.72 | 6 (27%) | 1 (5%) | 1 (14%) | 0 |
| Rituximab | 5 (10%) | 2 (9%) | 3 (10%) | 0.84 | 2 (9%) | 2 (10%) | 1 (14%) | 0 |
| Chemotherapy | 14 (27%) | 6 (26%) | 8 (28%) | 0.90 | 14 (64%) | 0 | 0 | 0 |
| Tocilizumab | 3 (6%) | 3 (13%) | 0 | 0.080 | 0 | 0 | 3 (43%) | 0 |
| Ruxolitinib | 1 (2%) | 1 (4%) | 0 | 0.44 | 0 | 0 | 3 (43%) | 0 |
| Overall mortality | 24 (46%) | 7 (30%) | 17 (59%) | 0.043 | 14 (64%) | 6 (30%) | 2 (29%) | 2 (100%) |
| Mortality 30 days | 13 (25%) | 3 (13%) | 10 (34%) | 0.076 | 9 (41%) | 3 (15%) | 0 | 1 (50%) |
| Mortality 90 days | 4 (10%) | 1 (4%) | 3 (16%) | 0,26 | 2 (15%) | 2 (12%) | 0 | 0 |
Notes: *Statistical analysis have been performed to compare each subgroup but is not presented in the table since the number of patients in MAS-HLH and idiopathic –HLH is lower and this give less value to a statistical analysis. Analysis of NK-cell activity, soluble CD25 (sIL-2R), were not performed in patients since these tests were not available on our center.
Abbreviations: ANC, absolute neutrophil count; Hb, hemoglobin; Pt, platelet; Fib, fibrinogen; TBIL, total bilirubin level; TG, triglycerides; ALAT, alanine aminotransferase; ASAT, aspartate aminotransferase; LDH, lactate dehydrogenase; M-HLH, malignancy-associated HLH; I-HLH, infection-associated HLH; MAS-HLH, macrophage activation syndrome; ULN, upper limits of normal.