Laparoscopic splenectomy prior to stem cell transplant for myelofibrosis with myeloid metaplasia may improve long-term survival
Emily E. Mackey, Richard Perugini
Surgery, University of Massachusetts Medical Center, Worcester, Massachusetts, United States
Myelofibrosis with myeloid metaplasia (MMM) is a rare chronic myeloproliferative disorder with a poor prognosis is associated with splenomegaly. Splenectomy has been associated with prohibitive morbidity, but offers the benefit of earlier engraftment after Stem cell transplant (SCT), a potential cure. We present a series of patients with MMM and splenomegaly that underwent laparoscopic splenectomy in preparation for SCT to examine operative morbidity, and whether splenectomy with SCT offers a survival benefit.
Prospective cohort study
Academic university hospital in a metropolitan setting.
This series of nine patients with MMM included 5 women and 4 men, with a median age of 69 years (range 34-81).
Main outcome measures
Actual survival compared to predicted survival; successful SCT
The median spleen size was 900 g (range 300-6620). Postoperative complications included deep venous thrombosis (1), hemorrhage (1), and infection (1), and atrial fibrillation (1). One perioperative mortality occurred due to leukemic transformation with pulmonary leukagglutination syndrome. The median length of stay was 5 (range 3-40) days. Seven (78%) were able to undergo SCT, a median of 82 days after splenectomy. The median survival was 373 days (range 15-2602). Four of nine patients met or exceeded the survival predicted by DIPSS score.
Laparoscopic splenectomy for MMM, in preparation for SCT, has a significant rate of morbidity. The minimally invasive technique does not appear to reduce morbidity of this procedure. However, splenectomy with SCT offers the possibility of long term survival for individuals with MMM with poor prognosis. A larger number of patients will need to be examined to fully understand the risks relative to the benefits.
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