Introduction
Methods
Patients and methods
Results
Clinical features
Case | Age | Sex | Cause | WBC (/μl) | ANC (/μl) | Hb (g/dl) | PLT (/μl) | AST (U/l) | ALT (U/l) | LDH (/UNL) | TG (mg/dl) | Fib (mg/dl) | CRP (mg/dl) |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
B1 | 17 | M | Unknown | 2,400 | 384 | 11.0 | 6.5 | 2,090 | 2,590 | 11.8 | n.t. | 198 | 0.5 |
B2 | 62 | F | Erythema exsudativum multiforme | 3,400 | 1,700 | 12.7 | 10.5 | 368 | 307 | 3.1 | 199 | 162 | 0.4 |
B3 | 52 | M | Pneumonia | 2,400 | 1,176 | 13.5 | 4.2 | 427 | 328 | 4.2 | n.t. | 415 | 19.9 |
B4 | 31 | M | CMV | 3,500 | 1,820 | 13.6 | 4.2 | 91 | 61 | 5.0 | n.t. | 478 | 23.6 |
B5 | 50 | F | Unknown | 600 | 189 | 11.2 | 5.4 | 6,300 | 3,400 | 32.3 | 157 | 134 | 7.4 |
B6 | 50 | M | EBV | 5,300 | 2,730 | 13.8 | 10.8 | 203 | 353 | 2.2 | n.t. | 333 | 3.1 |
B7 | 41 | M | Malaria | 4,100 | 1,353 | 10.4 | 7.6 | 60 | 73 | 2.0 | 729 | 574 | 14.2 |
B8 | 33 | M | Malaria | 2,600 | 1,300 | 10.7 | 4.3 | 40 | 74 | 1.4 | 310 | 456 | 19.8 |
B9 | 68 | F | AOSD | 2,400 | 984 | 9.2 | 13.9 | 57 | 37 | 1.4 | 123 | 276 | 1.2 |
B10 | 32 | F | AOSD | 13,800 | 12,765 | 11.6 | 4.3 | 1,978 | 1,820 | 17.5 | 239 | 188 | 3.8 |
Case | Splenomegaly | Hemophagocytosis (bone marrow) | NCC (/μl) | Histiocyte (%) | HLH-2004 | sIL2R (U/ml) | Ferritin (ng/ml) | sIL2R/ferritin |
---|---|---|---|---|---|---|---|---|
B1 | + | + | 65.0 × 104
| 0.6 | 5 (8) | 2,162 | 26,798 | 0.08 |
B2 | + | + | 3.0 × 104
| 1.6 | 5 (8) | 2,704 | 11,775 | 0.22 |
B3 | + | + | 8.6 × 104
| 6.0 | 5 (8) | 8,657 | 29,577 | 0.29 |
B4 | + | + | 7.0 × 104
| 2.6 | 5 (8) | 3,660 | 11,174 | 0.32 |
B5 | + | + | 2.1 × 104
| 9.6 | 7 (8) | 4,028 | 6,865 | 0.58 |
B6 | + | + | 3.0 × 104
| 1.0 | 5 (8) | 2,416 | 882 | 2.73 |
B7 | + | n.t. | n.t. | n.t. | 5 (8) | 2,563 | 6,947 | 0.37 |
B8 | + | n.t. | n.t. | n.t. | 5 (8) | 3,222 | 2,746 | 1.17 |
B9 | + | + | 0.9 × 104
| 3.0 | 5 (8) | 6,078 | 7,852 | 0.77 |
B10 | + | + | 6.0 × 104
| 3.4 | 5 (8) | 6,271 | 100,000 | 0.06 |
Case | Age | Sex | Cause | Diagnosed site | Bone marrow invasion | Stage | WBC (/μl) | ANC (/μl) | Hb (g/dl) | PLT (/μl) | AST (U/l) | ALT (U/l) | LDH (/UNL) | Fib (mg/dl) | TG (mg/dl) | CRP (mg/dl) |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
L1 | 73 | M | IVLBCL | Bone marrow | + | IVB | 4,100 | 3,342 | 10.8 | 7.6 | 25 | 19 | 1.7 | 213 | n.t. | 1.9 |
L2 | 67 | M | IVLBCL | Autopsy | + | IVB | 8,200 | 6,560 | 8.4 | 12.3 | 45 | 19 | 3.5 | 301 | 159 | 13.9 |
L3 | 98 | F | DLBCL, NOS | Lymph node | Not detected | IVB | 3,800 | 2,413 | 8.9 | 13.4 | 34 | 9 | 6.7 | n.t. | n.t. | 29.3 |
L4 | 74 | F | IVLBCL | Bone marrow | + | IVB | 4,200 | 1,953 | 7.6 | 16.6 | 58 | 25 | 2.5 | n.t. | n.t. | 17.3 |
L5 | 76 | M | DLBCL, NOS | Lymph node | + | IVB | 10,700 | 7,875 | 8.4 | 8.3 | 39 | 19 | 4.6 | 178 | n.t. | 9.0 |
L6 | 59 | F | IVLBCL | Skin, lung | Not detected | IVB | 4,900 | 2,793 | 9.0 | 20.5 | 46 | 8 | 8.9 | 408 | 306 | 8.2 |
L7 | 77 | F | IVLBCL | Bone marrow | + | IVB | 3,500 | 2,730 | 7.4 | 5.0 | 20 | 30 | 2.4 | 220 | n.t. | 3.0 |
L8 | 86 | F | IVLBCL | Bone marrow | + | IVB | 2,400 | 1,872 | 11.1 | 7.3 | 96 | 58 | 1.9 | 100 | 169 | 5.0 |
L9 | 56 | M | ENKL | Liver | + | IVB | 1,800 | 1,026 | 12.6 | 8.5 | 157 | 132 | 3.4 | 163 | 249 | 0.3 |
L10 | 62 | F | PTCL, NOS | Lymph node | + | IVB | 2,900 | 2,523 | 10.7 | 8.5 | 142 | 60 | 3.0 | 99 | 211 | 10.1 |
L11 | 65 | F | PTCL, NOS | Skin | + | IVB | 3,100 | 1,225 | 9.0 | 10.6 | 35 | 20 | 1.3 | 256 | n.t. | 1.1 |
Case | Splenomegaly | Hemophagocytosis | NCC (/μl) | Histiocyte (%) | HLH-2004 | sIL-2R (U/ml) | Ferritin (ng/ml) | sIL-2R/ferritin |
---|---|---|---|---|---|---|---|---|
L1 | + | + | 11.0 × 104
| 1.0 | 5 (8) | 9,221 | 2,613 | 3.52 |
L2 | + | + | 6.4 × 104
| 2.4 | 5 (8) | 9,793 | 3,273 | 2.99 |
L3 | + | + | Dry tap | 4.0 | 5 (8) | 12,569 | 2,861 | 4.39 |
L4 | + | + | 17.0 × 104
| 1.6 | 5 (8) | 4,291 | 1,729 | 2.48 |
L5 | + | + | 5.5 × 104
| 0.2 | 6 (8) | 36,938 | 1,184 | 31.19 |
L6 | + | + | 4.4 × 104
| 2.0 | 6 (8) | 3,462 | 718 | 4.82 |
L7 | + | + | 6.0 × 104
| 3.0 | 6 (8) | 11,536 | 707 | 16.31 |
L8 | + | + | 1.0 × 104
| 1.0 | 6 (8) | 36,715 | 3,477 | 10.55 |
L9 | + | + | 2.8 × 104
| 0.8 | 5 (8) | 3,588 | 4,923 | 0.72 |
L10 | + | + | 7.0 × 104
| 0.4 | 6 (8) | 10,230 | 6,067 | 1.68 |
L11 | + | + | 2.8 × 104
| 1.0 | 5 (8) | 9,620 | 619 | 15.54 |
Laboratory findings
Comparison of laboratory findings between LAHS and benign disease-associated HLH
Benign disease-associated HLH | Range | LAHS | Range |
p value | |
---|---|---|---|---|---|
Mean ± SD | Mean ± SD | ||||
n = 10 |
n = 11 | ||||
WBC (×109/l) | 4,050 ± 1,152 | 600–13,800 | 4,509 ± 799 | 1,800–10,700 | NS |
ANC (×109/l) | 2,440 ± 1,170 | 189–12,765 | 3,119 ± 650 | 1,026–7,875 | <0.05 |
Hb (g/dl) | 11.8 ± 0.5 | 9.2–13.8 | 9.4 ± 0.5 | 7.4–12.6 | <0.01 |
PLT (×109/l) | 7.2 ± 1.1 | 4.2–13.9 | 10.8 ± 1.4 | 5.0–20.5 | <0.05 |
AST (U/l) | 1,161 ± 622 | 40–6,300 | 63.4 ± 14.2 | 20–157 | <0.01 |
ALT (U/l) | 904 ± 391 | 37–3,400 | 36.3 ± 10.9 | 8–132 | <0.001 |
LDH (/UNL) | 8.1 ± 3.2 | 1.4–32.3 | 3.6 ± 0.7 | 1.3–8.9 | NS |
CRP (mg/dl) | 9.4 ± 2.9 | 0.4–23.6 | 9.0 ± 2.6 | 0.3–29.3 | NS |
sIL-2R (U/ml) | 4,176 ± 677 | 2,162–8,657 | 13,451 ± 3,611 | 3,462–36,938 | <0.005 |
Ferritin (ng/ml) | 20,462 ± 9,330 | 882–100,000 | 2,561 ± 544 | 619–6,067 | <0.005 |
sIL-2R/ferritin | 0.66 ± 0.25 | 0.06–2.73 | 8.56 ± 2.79 | 0.72–31.1 | <0.0005 |