Asunto(s)
Interferón-alfa/uso terapéutico , Venas Mesentéricas/patología , Policitemia Vera/complicaciones , Policitemia Vera/tratamiento farmacológico , Polietilenglicoles/uso terapéutico , Trombocitemia Esencial/complicaciones , Trombocitemia Esencial/tratamiento farmacológico , Trombosis de la Vena/etiología , Humanos , Interferón-alfa/administración & dosificación , Interferón-alfa/efectos adversos , Policitemia Vera/diagnóstico , Policitemia Vera/etiología , Polietilenglicoles/administración & dosificación , Polietilenglicoles/efectos adversos , Estudios Prospectivos , Proteínas Recombinantes/administración & dosificación , Proteínas Recombinantes/efectos adversos , Proteínas Recombinantes/uso terapéutico , Trombocitemia Esencial/diagnóstico , Trombocitemia Esencial/etiología , Trombosis de la Vena/diagnóstico , Trombosis de la Vena/terapiaRESUMEN
We retrospectively studied 181 patients with polycythaemia vera (n=67), essential thrombocythaemia (n=67) or primary myelofibrosis (n=47), who presented a first episode of splanchnic vein thrombosis (SVT). Budd-Chiari syndrome (BCS) and portal vein thrombosis were diagnosed in 31 (17.1%) and 109 (60.3%) patients, respectively; isolated thrombosis of the mesenteric or splenic veins was detected in 18 and 23 cases, respectively. After this index event, the patients were followed for 735 patient years (pt-years) and experienced 31 recurrences corresponding to an incidence rate of 4.2 per 100 pt-years. Factors associated with a significantly higher risk of recurrence were BCS (hazard ratio (HR): 3.03), history of previous thrombosis (HR: 3.62), splenomegaly (HR: 2.66) and leukocytosis (HR: 2.8). Vitamin K-antagonists (VKA) were prescribed in 85% of patients and the recurrence rate was 3.9 per 100 pt-years, whereas in the small fraction (15%) not receiving VKA more recurrences (7.2 per 100 pt-years) were reported. Intracranial and extracranial major bleeding was recorded mainly in patients on VKA and the corresponding rate was 2.0 per 100 pt-years. In conclusion, despite anticoagulation treatment, the recurrence rate after SVT in myeloproliferative neoplasms is high and suggests the exploration of new avenues of secondary prophylaxis with new antithrombotic drugs and JAK-2 inhibitors.
Asunto(s)
Síndrome de Budd-Chiari/fisiopatología , Policitemia Vera/fisiopatología , Mielofibrosis Primaria/fisiopatología , Trombocitemia Esencial/fisiopatología , Trombosis de la Vena/fisiopatología , Adulto , Anciano , Síndrome de Budd-Chiari/etiología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Policitemia Vera/complicaciones , Vena Porta/fisiopatología , Mielofibrosis Primaria/complicaciones , Modelos de Riesgos Proporcionales , Recurrencia , Estudios Retrospectivos , Factores de Riesgo , Trombocitemia Esencial/complicaciones , Trombosis de la Vena/etiologíaRESUMEN
The optimal duration of treatment with vitamin K antagonists (VKA) after venous thromboembolism (VTE) in patients with Philadelphia-negative myeloproliferative neoplasms (MPNs) is uncertain. To tackle this issue, we retrospectively studied 206 patients with MPN-related VTE (deep venous thrombosis of the legs and/or pulmonary embolism). After this index event, we recorded over 695 pt-years 45 recurrences, venous in 36 cases, with an incidence rate (IR) of 6.5 per 100 pt-years (95% confidence interval (CI): 4.9-8.6). One hundred fifty-five patients received VKA; the IR of recurrent thrombosis per 100 pt-years was 4.7 (95% CI: 2.8-7.3) on VKA and 8.9 (95% CI: 5.7-13.2) off VKA (P=0.03). In patients receiving VKA, the IR of recurrent thrombosis per 100 pt-years was 5.3 (95% CI: 3.2-8.4) among 108 patients on long-term VKA and 12.8 (95% CI: 7.3-20.7) after discontinuation among the 47 who ceased treatment (P=0.008), with a doubled risk of recurrence after stopping VKA (hazard ratio: 2.21, 95% CI: 1.19-5.30). The IR of major bleeding per 100 pt-years was 2.4 (95%: CI: 1.1-4.5) on VKA and 0.7 (95% CI: 0.08-2.5) off VKA (P=0.08). In conclusion, in MPN patients with VTE recurrent thrombosis is significantly reduced by VKA and caution should be adopted in discontinuation; however, the incidence of recurrence on treatment remains high, calling for clinical trials aimed to improve prophylaxis in this setting.