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Splenectomy and Risk of Blast Transformation in Myelofibrosis With Myeloid Metaplasia

Giovanni Barosi, Achille Ambrosetti, Antonietta Centra, Antonietta Falcone, Carlo Finelli, Paolo Foa, Alberto Grossi, Roberta Guarnone, Serena Rupoli, Luigiana Luciano, Maria C. Petti, Enrico Pogliani, Domenico Russo, Marco Ruggeri, and Silvana Quaglini for the Italian Cooperative Study Group on Myelofibrosis With Myeloid Metaplasia

From the Laboratorio di Informatica Medica and Dipartimento di Medicina Interna e Oncologia Medica, IRCCS Policlinico S. Matteo, Pavia, Italy; the Cattedra di Ematologia, Policlinico Borgo Roma, Verona, Italy; the Divisione di Ematologia, Ospedale S. Maria Goretti, Latina, Italy; the Divisione di Ematologia Casa Sollievo della Sofferenza, S. Giovanni Rotondo, Foggia, Italy; the Istituto di Ematologia "Seragnoli", Bologna, Italy; the Servizio di Ematologia Diagnostica, Istituto di Scienze Mediche, Milano, Italy; the Divisione di Ematologia, Policlinico di Carreggi, Firenze, Italy; the Clinica di Ematologia, Ospedale Generale Regionale, Torrette di Ancona, Italy; the Divisione di Ematologia, Nuovo Policlinico, Napoli, Italy; the Dipartimento di Biopatologia Umana, Università "La Sapienza", Roma, Italy; the Cattedra di Medicina Interna II, Nuovo Ospedale S. Gerardo, Monza, Italy; the Divisione di Ematologia, Policlinico Universitario, Udine, Italy; the Divisione di Ematologia, Ospedale S. Bortolo, Vicenza, Italy; and the Dipartimento di Informatica e Sistemistica, Università di Pavia, Pavia, Italy.

An unexpectedly high incidence of blast transformation after splenectomy has been reported in patients with myelofibrosis with myeloid metaplasia. However, whether this was associated with spleen removal after adjustment for risk factors was not determined. We conducted a multicenter historical cohort study of patients with myelofibrosis with myeloid metaplasia diagnosed from January 1970 through January 1994. A total of 549 patients (325 men and 224 women from 22 to 92 years of age; median age, 63 years) were included in the final data set. The Cox's proportional-hazards model was used to identify factors associated with blast transformation and death. To further adjust for factors related to spleen removal assignment, a propensity score for splenectomy was estimated using recursive-partitioning analysis. Blast transformation developed in 78 patients (14.2%). Patients who underwent splenectomy developed more blast transformations than those who were not splenectomized (23 of 87 [26.4%] v 55 of 462 [11.9%]; P < .001). The cumulative incidence of blast transformation 12 years after diagnosis was 27.0% in nonsplenectomized patients and 55.0% in splenectomized ones (P = .01). The risk factors independently predictive of blast transformation included prior splenectomy (relative risk = 2.61), platelet count less than 100 × 109/L at diagnosis (relative risk = 2.45), and the presence of blasts in peripheral blood at diagnosis (relative risk = 2.31). The relative risk of blast transformation in splenectomized patients increased from 2.2 at 48 months from diagnosis to 14.3 at 12 years. Patients with the same propensity score for splenectomy showed a higher risk for blast transformation on the basis of having undergone splenectomy (P = .02). In conclusion, the risk of blast transformation is significantly increased in subjects who underwent splenectomy and appears to be independent of factors related to spleen removal assignment.

Blood, Vol. 91 No. 10 (May 15), 1998: pp. 3630-3636
© 1998 by The American Society of Hematology.


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