Splenectomy in myelofibrosis with myeloid metaplasia: A single- institution experience with 223 patients

Ayalew Tefferi, Ruben Mesa, David M. Nagorney, Georgene Schroeder, Murray N. Silverstein

Research output: Contribution to journalArticle

219 Citations (Scopus)

Abstract

In a 20-year period, 223 patients (median age, 64.8 years) with myelofibrosis with myeloid metaplasia (MMM) had therapeutic splenectomy st 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 x 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. (C) 2000 by The American Society of Hematology.

Original languageEnglish (US)
Pages (from-to)2226-2233
Number of pages8
JournalBlood
Volume95
Issue number7
StatePublished - Apr 1 2000
Externally publishedYes

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Primary Myelofibrosis
Splenectomy
Lymphocyte Activation
Thrombocytopenia
Survival
Surgery
Thrombocytosis
Bone
Portal Hypertension
Anemia
Platelets
Bone Marrow
Liver
Splenomegaly
Platelet Count
Cytogenetics
Survivors
Thrombosis
Spleen
Morbidity

ASJC Scopus subject areas

  • Hematology

Cite this

Tefferi, A., Mesa, R., Nagorney, D. M., Schroeder, G., & Silverstein, M. N. (2000). Splenectomy in myelofibrosis with myeloid metaplasia: A single- institution experience with 223 patients. Blood, 95(7), 2226-2233.

Splenectomy in myelofibrosis with myeloid metaplasia : A single- institution experience with 223 patients. / Tefferi, Ayalew; Mesa, Ruben; Nagorney, David M.; Schroeder, Georgene; Silverstein, Murray N.

In: Blood, Vol. 95, No. 7, 01.04.2000, p. 2226-2233.

Research output: Contribution to journalArticle

Tefferi, A, Mesa, R, Nagorney, DM, Schroeder, G & Silverstein, MN 2000, 'Splenectomy in myelofibrosis with myeloid metaplasia: A single- institution experience with 223 patients', Blood, vol. 95, no. 7, pp. 2226-2233.
Tefferi A, Mesa R, Nagorney DM, Schroeder G, Silverstein MN. Splenectomy in myelofibrosis with myeloid metaplasia: A single- institution experience with 223 patients. Blood. 2000 Apr 1;95(7):2226-2233.
Tefferi, Ayalew ; Mesa, Ruben ; Nagorney, David M. ; Schroeder, Georgene ; Silverstein, Murray N. / Splenectomy in myelofibrosis with myeloid metaplasia : A single- institution experience with 223 patients. In: Blood. 2000 ; Vol. 95, No. 7. pp. 2226-2233.
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abstract = "In a 20-year period, 223 patients (median age, 64.8 years) with myelofibrosis with myeloid metaplasia (MMM) had therapeutic splenectomy st 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 x 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. (C) 2000 by The American Society of Hematology.",
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AB - In a 20-year period, 223 patients (median age, 64.8 years) with myelofibrosis with myeloid metaplasia (MMM) had therapeutic splenectomy st 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 x 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. (C) 2000 by The American Society of Hematology.

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