Risks and benefits of splenectomy in myelofibrosis with myeloid metaplasia: A retrospective analysis of 26 cases

Background and Objectives: To evaluate the outcomes of splenectomy in myelo fibrosis and myeloid metaplasia (MMM). Methods: We retrospectively reviewed our records of 26 patients with MMM wh o underwent an open splenectomy at Boston University Medical Center between 1979 and 1995. Fourteen patients had agnogenic myeloid metaplasia (AMM) an d 12 had myelofibrosis with antecedent myeloproliferative disorders (MF). T he main indications for splenectomy were progressive transfusion-dependent anemia, painful splenomegoaly, and hypercatabolic symptoms associated with cytopenia. Results: Median time to splenectomy after the diagnosis of MMM was 29 month s ranging from 1 to 96 months. Three patients (11%) died within I month aft er the surgery because of sepsis. The most common peri- and postoperative c omplications were pneumonia and other bacterial infections (42%): cardiac e vents (19%), acute bleeding (5%), ileus (15%), and venous thrombosis (12%). Of the eight surviving patients who underwent splenectomy for transfusion dependent anemia, six (75%) had improvement in their hematocrit levels with abolishment of blood transfusions. A durable symptomatic palliation was ac hieved in all patients. Liver enlargement was noted in seven patients at 1- year evaluation. None of these patients developed hepatic failure. Leukemic transformation occurred in 8 of 18 patients (44%) postsplenectomy. The med ian overall survival for the entire group was 58.5 and 28 months from the d iagnosis of MMM and the time of splenectomy, respectively. There was no dif ference in survival rates between patients with AMM and ME Conclusions: Splenectomy is an effective palliative procedure with an accep table morbidity in selected patients with MMM. Progressive transfusion-depe ndent anemia should also be considered an indication for splenectomy in the absence of leukemic evolution. (C) 2001 Wiley-Liss, Inc.