Table 1.
Characteristics of 18 sHLH patients
| No. | Age/ Sex | Underlying disease | Therapy | Outcome | Survival (month) | Organs | SUV max |
|---|---|---|---|---|---|---|---|
| 1 |
35/M |
Lymphoma (NK / T) |
IVIG/HLH-2004 regimen(1 cycle) → High-dose methylprednisolone pulse therapy |
Died of intracranial hemorrhage |
1.7 |
6 |
12.3 |
| 2 |
35/F |
Lymphoma (NK / T) |
The Hyper-CVAD regimen (1 cycle) |
Died of intracranial hemorrhage |
1.2 |
6 |
15.7 |
| 3 |
18/M |
Lymphoma (NK / T) |
The CHOP regimen(1 cycle) |
Died of acute hemorrhage of gastrointestinal tract |
0.3 |
7 |
14.6 |
| 4 |
56/M |
Lymphoma |
Hydrocortisone 100mg×5d |
Died of intracranial hemorrhage |
1.7 |
5 |
4.3 |
| 5 |
32/M |
Lymphoma |
Dex 10mg/d×3d |
Died of liver failure |
0.3 |
10 |
13.3 |
| 6 |
37/F |
Sjögren's syndrome |
The COP regimen(3 cycle) |
CR |
>12 |
5 |
0.7 |
| 7 |
15/F |
UCTD |
The COP regimen (4 cycle) |
CR |
>45 |
3 |
4.6 |
| 8 |
21/F |
EBV infection |
HLH-2004 regimen (1 cycle) |
Died of acute hemorrhage of gastrointestinal tract |
1.7 |
7 |
6.6 |
| 9 |
17/M |
EBV infection |
Methylprednisolone 40 mg/d×24d |
CR |
>22 |
7 |
8.3 |
| 10 |
46/M |
EBV infection |
Dex 15mg/d×4d |
Died of septic shock |
0.4 |
6 |
10 |
| 11 |
73/M |
EBV infection |
The COP regimen (7 cycle) |
Died of multi-organ failure |
6 |
7 |
5.2 |
| 12 |
26/F |
CMV infection |
IVIG/HLH-2004 regimen (1 cycle) →The CHOP regimen(2 cycle) |
CR |
>24 |
6 |
9 |
| 13 |
24/F |
CMV infection |
The COP regimen (7 cycle) |
Died of respiratory failure |
2.2 |
5 |
4.2 |
| 14 |
69/F |
MRSH infection |
The COP regimen (2 cycle) |
Died of respiratory failure |
2.0 |
6 |
5.2 |
| 15 |
62/F |
Fungal Infection |
The COP regimen (7 cycle) |
stable |
>8 |
4 |
5.8 |
| 16 |
44/F |
Malignant tumour? |
Methylprednisolone 40 mg/d×5d |
Died of multi-organ failure |
0.4 |
8 |
7.7 |
| 17 |
56/M |
Lymphoma? |
The CHOP regimen (2 cycle) →Splenectomy→The Hyper-CVAD regimen (1cycle) |
stable |
>13 |
6 |
5.7 |
| 18 | 18/M | indefinite | HLH-2004 regimen (1 cycle) | Died of intracranial hemorrhage | 0.2 | 3 | 4.2 |
HLH-2004, dexamethasone, etopside and Ciclosporin A; CHOP, cyclophosphamide, adviamycin, vincristine and prednisolone; COP, cyclophosphamide, vincristine and prednisone; Hyper-CVAD, cyclophosphamide, vincristine, doxorubicin, dexamethasone, methotrexate and cytarabine; DEX, dexamethasone; CR, complete response; UCTD, undifferentiated connective tissue disease; EBV, Epstein-Barr virus; CMV, cytomegalovirus; MRSH, methicillin-resistant Staphylococcus hominis.