Outcome of Immunochemotherapy in 17 Patients With EBV-HLH
| Case No. . | Induction Regimen . | Initial Response at 8 Weeks . | Continuation Regimen . | Total Duration of Therapy (mo) . | Final Response . | CR Duration (mo) . | Survival (mo) . |
|---|---|---|---|---|---|---|---|
| 1 | PE, VCR/VP16/PSL/CSA* | PR | HLH-94 | 4+ | CR | 4+ | 8+ |
| 2 | IVIG/HLH-94 | PR | BMT | 2 | CR | 7+ | 9+ |
| 3 | HLH-94 | CR | None | 2 | CR | 8+ | 10+ |
| 4 | ET, HLH-94/CSA* | PR | HLH-94 | 11+ | CR/REL | 6‡ | 11+ |
| 5 | HLH-94 | CR/REL | HLH-94 | 7 | CR | 11+ | 14+ |
| 6 | PE, VP16/PSL/MTX | PR | VP16/PSL/MTX | 5 | CR | 15+ | 19+ |
| 7 | IVIG/VP16/PSL | PR | VP16/PSL | 12 | CR | 14+ | 18+ |
| 8 | HLH-94 | CR | None | 2 | CR | 15+ | 17+ |
| 9 | IVIG/PSL/VP16 | PR | HLH-94 | 12 | CR | 17+ | 21+ |
| 10 | HLH-94† | PR | HLH-94 | 12 | CR | 15+ | 23+ |
| 11 | HLH-94 | PR | HLH-94 | 9 | CR | 19+ | 25+ |
| 12 | VP16/VCR/PSL | CR | None | 1 | CR | 22+ | 24+ |
| 13 | ET, HLH-94 | PR/REL | NHL-chemo/CSA/BMT | 7 | CR | 19+ | 25+ |
| 14 | PE, VP16/HLH-94 | PR | HLH-94 | 9 | CR | 30+ | 36+ |
| 15 | PSL/IVIG, VP16/PSL | PR | NHL-chemo | 36 | CR | 39+ | 45+ |
| 16 | PE, VP16/PSL | PR/REL | NHL/HD-chemo/CSA | 27 | CR | 37+ | 62+ |
| 17 | PE, mPSL/Lipo-Dexa/CSA* | CR | None | 2 | CR | 5+ | 7+ |
| Case No. . | Induction Regimen . | Initial Response at 8 Weeks . | Continuation Regimen . | Total Duration of Therapy (mo) . | Final Response . | CR Duration (mo) . | Survival (mo) . |
|---|---|---|---|---|---|---|---|
| 1 | PE, VCR/VP16/PSL/CSA* | PR | HLH-94 | 4+ | CR | 4+ | 8+ |
| 2 | IVIG/HLH-94 | PR | BMT | 2 | CR | 7+ | 9+ |
| 3 | HLH-94 | CR | None | 2 | CR | 8+ | 10+ |
| 4 | ET, HLH-94/CSA* | PR | HLH-94 | 11+ | CR/REL | 6‡ | 11+ |
| 5 | HLH-94 | CR/REL | HLH-94 | 7 | CR | 11+ | 14+ |
| 6 | PE, VP16/PSL/MTX | PR | VP16/PSL/MTX | 5 | CR | 15+ | 19+ |
| 7 | IVIG/VP16/PSL | PR | VP16/PSL | 12 | CR | 14+ | 18+ |
| 8 | HLH-94 | CR | None | 2 | CR | 15+ | 17+ |
| 9 | IVIG/PSL/VP16 | PR | HLH-94 | 12 | CR | 17+ | 21+ |
| 10 | HLH-94† | PR | HLH-94 | 12 | CR | 15+ | 23+ |
| 11 | HLH-94 | PR | HLH-94 | 9 | CR | 19+ | 25+ |
| 12 | VP16/VCR/PSL | CR | None | 1 | CR | 22+ | 24+ |
| 13 | ET, HLH-94 | PR/REL | NHL-chemo/CSA/BMT | 7 | CR | 19+ | 25+ |
| 14 | PE, VP16/HLH-94 | PR | HLH-94 | 9 | CR | 30+ | 36+ |
| 15 | PSL/IVIG, VP16/PSL | PR | NHL-chemo | 36 | CR | 39+ | 45+ |
| 16 | PE, VP16/PSL | PR/REL | NHL/HD-chemo/CSA | 27 | CR | 37+ | 62+ |
| 17 | PE, mPSL/Lipo-Dexa/CSA* | CR | None | 2 | CR | 5+ | 7+ |
Abbreviations: EBV-HLH, Epstein-Barr virus-related hemophagocytic lymphohistiocytosis; PE, plasma exchange; ET, exchange transfusion; VCR, vincristine; VP16, etoposide; PSL, prednisolone; CSA, cyclosporin A; IVIG, intravenous immunoglobulin; HLH-94, HLH treatment protocol developed by investigators of the Histiocyte Society; MTX, methotrexate; mPSL, methyl-prednisolone; Lipo-Dexa, dexamethasone in lipid microspheres; PR, partial response; CR, complete response; REL, relapse; NHL-chemo, chemotherapy as given to NHL patients37; HD-chemo, chemotherapy as given to HD patients38; BMT, bone marrow transplantation.
Short-term continuous infusion.
With four intrathecal MTX injections.
Await for BMT after relapse.