24 November 2025 : Clinical Research
Perforin Expression and Natural Killer-Cell Proportion as Biomarkers in Secondary Hemophagocytic Lymphohistiocytosis
Jujuan Wang EF 1, Xin Li BE 2, Limin Duan BC 1, Guangli Yin CG 1, Xin Gao AF 1, Hongxia Qiu G 1, Ji Xu AD 1*, Tian Tian
AB 1
DOI: 10.12659/MSM.950615
Med Sci Monit 2025; 31:e950615
Table 1 Clinical data of 19 patients initially diagnosed with secondary hemophagocytic lymphohistiocytosis (sHLH).
| No. | Age/sex | Diagnosis | Treatment | Overall survival (months) | Treatment response | Prognosis |
|---|---|---|---|---|---|---|
| 1 | 66/M | LHLH | CHOP | 2.3 | NR | Died |
| 2 | 65/M | LHLH | High-dose corticosteroid & IVIG+antiviral therapy +3 cycle MINE | 39.7 | PR | Died |
| 3 | 27/M | LHLH | HLH-2004 +3 cycle MINE +3 cycle CHOPE | 93.0 | CR | Survival |
| 4 | 59/M | LHLH | 3 cycle MINE + splenic radiotherapy +3 cycle DHAP | 39.9 | PR | Died |
| 5 | 45/M | LHLH | HLH-2004 +2 cycle MINE +1 cycle GDP +1 cycle HyperCVAD-A +1 cycle CHOP | 6.0 | PR | Died |
| 6 | 30/F | LHLH | HLH-2004 +3 cycle MINE +1 cycle DHAP +1 cycle HyperCVAD-A | 17.1 | PR | Died |
| 7 | 60/M | LHLH | SMILE | 0.9 | NR | Died |
| 8 | 41/F | LHLH | High-dose corticosteroid & IVIG + HLH-2004 +12 cycle L-GemOx | 92.3 | CR | Survival |
| 9 | 60/M | LHLH | 4 cycle CHOP +4 cycle GDP | 7.8 | PR | Died |
| 10 | 59/M | LHLH | High-dose corticosteroid & IVIG+ HLH-2004 +8 cycle MINE | 98.0 | CR | Survival |
| 11 | 37/M | LHLH | High-dose corticosteroid & IVIG +2 cycle MINE+ L-GemOx +3cycle GemOx | 7.3 | PR | Died |
| 12 | 39/M | IHLH (EBV, fungus) | High-dose corticosteroid & IVIG+HLH-2004+ anti-fungal theraphy | 96.0 | CR | Survival |
| 13 | 57/M | IHLH (fungus) | Anti-fungal therapy + anti-infectious therapy + corticosteroid | 93.0 | CR | Survival |
| 14 | 59/M | IHLH (pneumonia) | Supportive treatment | NA | NA | Lost to follow-up |
| 15 | 29/F | IHLH (EBV) | Corticosteroid + antiviral therapy + supportive treatment | 2.9 | NR | Died |
| 16 | 52/F | IHLH (sepsis) | Anti-infectious therapy + supportive treatment | 94.1 | CR | Survival |
| 17 | 48/M | IHLH (sepsis) | High-dose corticosteroid & IVIG+ anti-infectious therapy | 104.0 | CR | Survival |
| 18 | 58/F | AHLH | COP+ High-dose corticosteroid & IVIG&CTX | 3.1 | NR | Died |
| 19 | 46/M | AHLH | COP+ supportive treatment | 12.0 | PR | Died |
| F – Female; M – Male; AHLH – rheumatic immune-associated hemophagocytic lymphohistiocytosis; IHLH – infection-associated hemophagocytic lymphohistiocytosis; LHLH – lymphoma-associated hemophagocytic lymphohistiocytosis; OS – overall survival; CHOP – cyclophosphamide, doxorubicin, vincristine, prednisolone; IVIG – intravenous immunoglobulin; MINE – mitoxantrone, ifosfamide, mesna, etoposide; HLH-2004 – dexamethasone, etoposide, cyclosporine A; DHAP – dexamethasone, cisplatin, cytosine arabinoside; GDP – gemcitabine, dexamethasone, cisplatin; Hyper-CVAD-A – cyclophosphamide, mesna, vincristine, doxorubicin, dexamethasone; SMILE – dexamethasone, methotrexate, ifosfamide, L-asparaginase, etoposide; GemOx – gemcitabine, oxaliplatin; L-GemOx – L-asparaginase, gemcitabine, oxaliplatin; COP – cyclophosphamide, vincristine, prednisolone; CR – complete response; PR – partial response; NR – no response; NA – not applicable. | ||||||