Sequential treatment of hairy cell leukemia: a new role for interferon

One must consider the need for treatment in each patient with HCL individually. A small minority of patients need no treatment and have a long survival. We continue to recommend splenectomy as the initial therapy in patients with cytopenias or splenic complications; however, the initial management of leukemic patients is less well defined because splenectomy will not decrease the WBC and frequently will exacerbate the hyperleukocytosis. This group of patients might benefit from initial IFN therapy, although this has not been established in studies to date. Management of patients with advanced disease postsplenectomy is less controversial at this time. IFN has been demonstrated to be a safe, highly effective palliative treatment by multiple investigators. Chemotherapy should be avoided in patients with pancytopenia, although it may be used as part of an overall treatment plan in conjunction with IFN. Further trials are indicated to determine whether late intensification or "adjuvant" chemotherapy will be advantageous in partial and complete responders to IFN therapy. With multiple treatment strategies and careful attention to developing complications, survival in patients with hairy cell leukemia should continue to improve for newly diagnosed patients. Based on the use of some of these strategies in 199 patients that we have identified since 1974, we can show that the survival is good and that the median actuarial survival has not yet been reached (Fig. 15-4).