Bone Marrow Transplantation

Allogeneic bone marrow transplantation (BMT) has recently been shown to be a promising therapeutic approach, which results in complete hematological remission in the majority of cases and long-term survival in a substantial number of patients.31 The 5-year overall survival of 54 percent for individuals receiving an unmanipulated HLA-matched related graft is particularly encouraging. Splenectomy before transplantation may reduce the risk of delayed engraftment and is recommended in patients with marked splenomegaly. Nevertheless, treatment failure occurs in approximately one quarter of cases and is associated with older age, presence of an abnormal kary-otype, and the absence of grade II to IV acute graft-versus-host disease. It would seem reasonable, therefore, to offer allogeneic BMT to young patients with a poor life expectancy (as defined above), although the timing of BMT in patients with a favorable prognosis remains a dilemma. Donor lymphocyte infusions have been reported to induce remission of relapsed disease following allogeneic BMT,32 a finding that supports the concept of a "graft-versus-myelofibrosis" effect. The latter observation also provides a basis for designing new therapeutic strategies such as the nonmyeloablative or "mini" transplant approach, which could extend the upper age limit for transplantation. Autolo-gous BMT, despite the reinfusion of clonal peripheral blood stem cells, can reduce marrow fibrosis, restore hematopoiesis, and result in an overall improvement in quality of life.33 Although this is not a curative procedure, we would suggest that peripheral stem cells be harvested at diagnosis and that an autologous BMT be considered in those individuals with advancing disease in whom an allo-BMT is not feasible.

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