Factor XI deficiency in Iranians: its clinical manifestations in comparison with those of classic hemophilia

BACKGROUND AND OBJECTIVES: Patients affected by Hodgkin's disease (HD) resistant to induction therapy or who have a brief duration of first remission have a poor outcome. DESIGN AND METHODS: We retrospectively reviewed the clinical data of 28 patients affected by Hodgkin's disease who relapsed 6 to 24 months from completion of treatment (14 patients) or who were refractory to first-line therapy or relapsed very early (14 patients). All the 28 patients were treated with salvage chemotherapy plus a conditioning regimen followed by peripheral blood stem cell transplant (PBCST) or autologous bone marrow transplant (ABMT). RESULTS: At a median follow-up of 35.5 months (range 14-119), of the 14 patients responding to first-line therapy but who relapsed > 6 months off therapy, 10 (72%) are alive, well and in complete remission (CR), 2 (14%) are alive with disease at 39 and 83 months from transplant, and 2 (14%) died 26 and 63 months after their transplant from acute myeloid leukemia and HD, respectively. At a median follow-up of 39 months, the overall survival (OS) is 68% and the event-free survival (EFS) is 56%. At a median follow-up of 30 months (1-98), of the 14 patients refractory to first-line therapy or who relapsed very early, 9 (64%) are alive in CR, 1 (7%) is alive with disease and 4 (29%) have died of their disease (3 patients) or myelodysplastic syndrome (1 patient). The OS is 58% and the EFS is 52%. There are no statistically significant differences in terms of OS and EFS between the two groups of patients. INTERPRETATION AND CONCLUSIONS: Our study shows that salvage chemotherapy followed by a conditioning regimen and autotransplant is an effective, feasible and well-tolerated scheme of therapy not only for patients with HD who relapse after first-line treatment, but also for those resistant to first-line treatment.