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Blood, Vol. 95 No. 7 (April 1), 2000:
pp. 2226-2233
Splenectomy in myelofibrosis with myeloid metaplasia: a
single-institution experience with 223 patients
Ayalew Tefferi,
Ruben A. Mesa,
David M. Nagorney,
Georgene Schroeder, and
Murray N. Silverstein
From the Division of Hematology and Internal Medicine, the Division
of Gastroenterologic and General Surgery, and the Cancer Center
Statistics Unit, Mayo Clinic and Mayo Foundation, Rochester, MN.
In a 20-year period, 223 patients (median age, 64.8 years) with
myelofibrosis with myeloid metaplasia (MMM) had therapeutic splenectomy at our institution. Primary indications for surgery were
transfusion-dependent anemia (45.3%), symptomatic splenomegaly (39.0%), portal hypertension (10.8%), and severe thrombocytopenia (4.9%). Operative mortality and morbidity rates were 9% and 31%, respectively. The 203 survivors of surgery had a median postsplenectomy survival time (PSS) of 27 months (range, 0-155). Among preoperative variables, thrombocytopenia (platelet count less than 100 × 109/L) and nonhypercellular bone marrow were identified as
independent risk factors for decreased PSS. Durable remissions in
constitutional symptoms, transfusion-dependent anemia, portal
hypertension, and severe thrombocytopenia were achieved in 67%, 23%,
50%, and 0% of the patients, respectively. Histologic or cytogenetic
features of bone marrow obtained before splenectomy did not predict a
response in cytopenias. After splenectomy, substantial enlargement of
the liver and marked thrombocytosis occurred in 16.1% and 22.0% of the patients, respectively. The thrombocytosis was associated with an
increased risk of perioperative thrombosis and decreased PSS. The rate
of blast transformation (BT) was 16.3%, and the risk of BT was higher
in the presence of increased spleen mass and preoperative
thrombocytopenia. However, the PSS of patients with BT was not
significantly different from that of patients without BT. We conclude
that presplenectomy thrombocytopenia in MMM may be a surrogate for
advanced disease and is associated with an increased risk of BT and
inferior PSS. However, the development of BT after splenectomy may not
affect overall survival and does not undermine the palliative role of
the procedure for the other indications.
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