Abstract
Introduction: HSCT has been developed in few Wm cases and is nowadays challenged by other innovative approaches. However, high dose therapy followed by autologous HSCT (HD-auto) produces high response rate and some long term responses while allogeneic HSCT performed after either myeloablative (MA-allo) or reduced intensity conditioning (RIC-allo) regimens may be cure of Wm (Dreger 1998, Tournilhac 2003, Maloney 2006).
Methods: We updated and extended our retrospective experience on 32 HD-auto, 11 MA-allo and 11-RIC-allo performed from 1990 to 2006 in 51 patients from 18 institutions. A MA-allo and a RIC-allo were performed in 1 and 2 cases respectively following relapse after a 1st HD-auto.
Results: Data are presented in the table.
| . | HD-auto . | MA-allo . | RIC-allo . |
|---|---|---|---|
| Nb | 32 | 11 | 11 |
| Median age at transplant | 56 | 46 | 56 |
| Interval: diagnosis-transplant | 38 | 50 | 74 |
| Chemoresistance at transplant | 25% | 36% | 55% |
| Conditioning regimen | BEAM (13), TBI/melphalan (9), TBI/endoxan (7), other (3) | TBI/endoxan (9), other (2) | TBI/fluda (10), other (1) |
| Donor | Sibling (9), unrelated (2) | Sibling (8), unrelated (2), cord blood (1) | |
| Median follow up (m) | 45 (3–121) | 68 (3–132) | 22(2–60) |
| Relapse | 18 (56%) | 4 (36%) | 0 |
| Transplant related mortality | 12,5% (one 2th cancer) | 36% | 27% (one 2th cancer) |
| overall survival (1;3;5y) | 87%, 77%, 58% | 64%, 54%, 54% | 82%, 68%, 68% |
| Event free survival (5y) | 25% | 48% | 68% |
| . | HD-auto . | MA-allo . | RIC-allo . |
|---|---|---|---|
| Nb | 32 | 11 | 11 |
| Median age at transplant | 56 | 46 | 56 |
| Interval: diagnosis-transplant | 38 | 50 | 74 |
| Chemoresistance at transplant | 25% | 36% | 55% |
| Conditioning regimen | BEAM (13), TBI/melphalan (9), TBI/endoxan (7), other (3) | TBI/endoxan (9), other (2) | TBI/fluda (10), other (1) |
| Donor | Sibling (9), unrelated (2) | Sibling (8), unrelated (2), cord blood (1) | |
| Median follow up (m) | 45 (3–121) | 68 (3–132) | 22(2–60) |
| Relapse | 18 (56%) | 4 (36%) | 0 |
| Transplant related mortality | 12,5% (one 2th cancer) | 36% | 27% (one 2th cancer) |
| overall survival (1;3;5y) | 87%, 77%, 58% | 64%, 54%, 54% | 82%, 68%, 68% |
| Event free survival (5y) | 25% | 48% | 68% |
Acute GVHD developed following 9 MA-allo [Grade III-IV (n=1)] and 8 RIC-allo [Grade III-IV (n=1)]. Chronic GVHD developed following 7 MA-allo [limited (n=5), extensive (n=2)] and 5 RIC-allo [limited (n=2), extensive (n=3)].
Conclusion: We confirm that autologous HSCT achieves some long term responses even in heavily pretreated patients. Allogeneic HSCT induces very long term disease control and may cure WM. Specially, the RIC-allo gives impressive results on disease control in a set of older patients, with refractory disease, mostly heavily pretreated.
Author notes
Disclosure: No relevant conflicts of interest to declare.
