title: Spleen enlargement is a risk factor for thrombosis in essential thrombocythemia: Evaluation on 1,297 patients.
abstract: Spleen enlargement, present in 10-20% of Essential Thrombocythemia (ET) patients at diagnosis, is a feature clinically easy to assess, confirmable by echography with a very low chance of misinterpretation. Nonetheless, the clinical and prognostic role of splenomegaly has been seldom evaluated. From 1979 to 2013, 1297 ET patients retrospectively collected in the database of the Lazio Cooperative Group and Bologna University Hospital were evaluable for spleen enlargement at diagnosis and included in the analysis. On the whole, spleen was enlarged in 172/1297 (13.0%) patients; in most cases (94.8%) splenomegaly was mild (≤5 cm). Patients with splenomegaly were younger, predominantly male, presented higher platelet count and JAK2V617F allele burden and had a lower incidence of concomitant cardiovascular risk factors. At least one thrombotic event during follow-up occurred in 97/1,125 (8.6%) patients without spleen enlargement compared to 27/172 (15.7%) patients with spleen enlargement (P = 0.003). Despite comparable use of cytoreductive/antiplatelet therapies in the two groups, the cumulative risk of thrombosis at 5 years was significantly higher in patients with baseline splenomegaly (9.8% versus 4.4% in patients without splenomegaly, P = 0.012). In multivariate analysis exploring risk factors for thrombosis, splenomegaly retained its negative prognostic role, together with previous thrombosis, leucocyte count and male gender. Baseline splenomegaly seems to be an independent additional risk factor for thrombosis in nonstrictly WHO-defined ET patients. This data could be useful in the real-life clinical management of these patients.
authors.affiliation: Division of Hematology, Nuovo Regina Margherita Hospital, Rome.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Andriani
authors.affiliation: Department of Cellular Biotechnologies and Hematology, University "La Sapienza", Rome.
authors.forename: Roberto
authors.initials: R
authors.lastname: Latagliata
authors.affiliation: San Giovanni Hospital, Divison of Hematology, Rome.
authors.forename: Barbara
authors.initials: B
authors.lastname: Anaclerico
authors.affiliation: Hematology and Stem Cell Transplantation Unit, Regina Elena National Cancer Institute, Rome.
authors.forename: Antonio
authors.initials: A
authors.lastname: Spadea
authors.affiliation: Division of Hematology, Polo Universitario Pontino, Latina.
authors.forename: Angela
authors.initials: A
authors.lastname: Rago
authors.affiliation: Division of Hematology, University "Tor Vergata", Rome.
authors.forename: Ambra
authors.initials: A
authors.lastname: Di Veroli
authors.affiliation: Division of Hematology and Bone Marrow Transplantation, Azienda Ospedaliera San Camillo-Forlanini, Rome.
authors.forename: Francesca
authors.initials: F
authors.lastname: Spirito
authors.affiliation: Division of Hematology, Sant'andrea Hospital, Rome.
authors.forename: Raffaele
authors.initials: R
authors.lastname: Porrini
authors.affiliation: Hematology, University "Campus Biomedico", Rome.
authors.forename: Marianna
authors.initials: M
authors.lastname: De Muro
authors.affiliation: Division of Hematology, SandroPertini Hospital, Rome.
authors.forename: Sabrina
authors.initials: S
authors.lastname: Crescenzi Leonetti
authors.affiliation: Division of Hematology, Nuovo Regina Margherita Hospital, Rome.
authors.forename: Nicoletta
authors.initials: N
authors.lastname: Villivà
authors.affiliation: Hematology, Belcolle Hospital, Viterbo.
authors.forename: Cinzia
authors.initials: C
authors.lastname: De Gregoris
authors.affiliation: Division of Hematology, Sant'andrea Hospital, Rome.
authors.forename: Enrico
authors.initials: E
authors.lastname: Montefusco
authors.affiliation: Institute of Hematology "L. And a. Seràgnoli", Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, S. Orsola-Malpighi Hospital, Italy.
authors.forename: Nicola
authors.initials: N
authors.lastname: Polverelli
authors.affiliation: Department of Cellular Biotechnologies and Hematology, University "La Sapienza", Rome.
authors.forename: Cristina
authors.initials: C
authors.lastname: Santoro
authors.affiliation: Department of Cellular Biotechnologies and Hematology, University "La Sapienza", Rome.
authors.forename: Massimo
authors.initials: M
authors.lastname: Breccia
authors.affiliation: IFO-San Gallicano Hospital, UOC Of Hematology, Rome.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Cimino
authors.affiliation: Division of Hematology and Bone Marrow Transplantation, Azienda Ospedaliera San Camillo-Forlanini, Rome.
authors.forename: Ignazio
authors.initials: I
authors.lastname: Majolino
authors.affiliation: Department of Cellular Biotechnologies and Hematology, University "La Sapienza", Rome.
authors.forename: Maria Gabriella
authors.initials: MG
authors.lastname: Mazzucconi
authors.affiliation: Institute of Hematology "L. And a. Seràgnoli", Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, S. Orsola-Malpighi Hospital, Italy.
authors.forename: Nicola
authors.initials: N
authors.lastname: Vianelli
authors.affiliation: Department of Cellular Biotechnologies and Hematology, University "La Sapienza", Rome.
authors.forename: Giuliana
authors.initials: G
authors.lastname: Alimena
authors.affiliation: Hematology, Belcolle Hospital, Viterbo.
authors.forename: Marco
authors.initials: M
authors.lastname: Montanaro
authors.affiliation: Institute of Hematology "L. And a. Seràgnoli", Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, S. Orsola-Malpighi Hospital, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Palandri
mesh_terms: D000328:Adult; D000368:Aged; D016208:Databases, Factual; D005260:Female; D006801:Humans; D053614:Janus Kinase 2 / Q000235:genetics; D008297:Male; D008875:Middle Aged; D009154:Mutation; D010975:Platelet Aggregation Inhibitors / Q000008:administration & dosage / Q000627:therapeutic use; D010976:Platelet Count; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D013163:Splenomegaly / Q000150:complications* / Q000000981:diagnostic imaging / Q000453:epidemiology; D013920:Thrombocythemia, Essential / Q000150:complications* / Q000000981:diagnostic imaging / Q000453:epidemiology; D013927:Thrombosis / Q000453:epidemiology / Q000209:etiology* / Q000517:prevention & control; D014463:Ultrasonography
chemical_list: D010975:Platelet Aggregation Inhibitors; C507924:JAK2 protein, human; D053614:Janus Kinase 2