Table 3.
Characteristics of Studies on Salvage Therapy for Refractory/Relapsed Adult HLH
| Treatment | Number of Patients | Type of HLH | Complete Response/Partial Response | Overall Response | Number of Patients Who Achieved HSCT or Subsequent Chemotherapy | Rate of Survival |
|---|---|---|---|---|---|---|
| Alemtuzumab (Marsh, et al)45 | 22 | 8 F-HLH | 0%/64% | 64% | 77% | 64% |
| 5 EBV-HLH | ||||||
| 2 CMV-HLH | ||||||
| 7 I-HLH | ||||||
| DEP (Wang, et al)44 | 63 | 29 Mal-HLH | 27%/49.2% | 76.2% | 13 (44.8%) | 60.4% |
| 22 EBV-HLH | ||||||
| 4 F-HLH | ||||||
| 8 I-HLH | ||||||
| L-DEP (Wang, et al)53 | 28 | EBV-HLH | 32%/53.5% | 85.7% | 13 (54.1%) | 76.9% (post HSCT) |
| Ruxolitinib (Ahmed, et al)60 | 5 | Secondary HLH | 100% | 100% | NA | 100% |
| Ruxolitinib (Boonstra, et al)61* | 13 | 5 Infectious-HLH | 38.4%/38.4% | 77% | NA | 76.9% |
| 4 MAS-HLH | ||||||
| 5 I-HLH | ||||||
| Ruxolitinib with and without glucocorticoids (Wang, et al)59 | 34 | 1 F-HLH | 14.7%/58.8% | 73.5 | NA | 55.9% |
| 25 EBV-HLH | ||||||
| 2 MAS | ||||||
| 6 I-HLH | ||||||
| DEP-Ru (Wang, et al)62 | 54 | 28 EBV-HLH | 15.1%/58.5% | 73.6% | 32 (59.2%) | NA |
| 5 MAS | ||||||
| 3 Mal-HLH | ||||||
| 6 F-HLH | ||||||
| 1 Pregnancy-HLH | ||||||
| 1 Drug-HLH | ||||||
| 2 Infect-HLH | ||||||
| Emapalumab (Locatelli, et al)55 | 34 | 27 F-HLH | 21%/32% | 65% | 22 (64.7%) | 69% (90.9% post HSCT) |
| 7 I-HLH | ||||||
| Nivolumab (Liu, et al)64 | 7 | EBV-HLH | 71.4%/14.2% | 85.6% | NA | 71.4% |
Note: *This study is an extension of Ahmed, et al study (Lancet Haematol 2019) but with data on longer follow-up and 7 more patients.
Abbreviations: NA, not available; DEP, doxorubicin, etoposide, methylprednisolone; L-DEP, DEP regimen with PEG aspargase; DEP-Ru, DEP-ruxolitinib; EBV, Epstein-Barr virus; CMV, cytomegalovirus; Mal-HLH, malignancy related HLH, I-HLH, idiopathic HLH; F-HLH, familial HLH; MAS, macrophage-activation syndrome; HSCT, hematopoietic stem-cell transplantation.