Subject(s)
Interferon-alpha/therapeutic use , Mesenteric Veins/pathology , Polycythemia Vera/complications , Polycythemia Vera/drug therapy , Polyethylene Glycols/therapeutic use , Thrombocythemia, Essential/complications , Thrombocythemia, Essential/drug therapy , Venous Thrombosis/etiology , Humans , Interferon-alpha/administration & dosage , Interferon-alpha/adverse effects , Polycythemia Vera/diagnosis , Polycythemia Vera/etiology , Polyethylene Glycols/administration & dosage , Polyethylene Glycols/adverse effects , Prospective Studies , Recombinant Proteins/administration & dosage , Recombinant Proteins/adverse effects , Recombinant Proteins/therapeutic use , Thrombocythemia, Essential/diagnosis , Thrombocythemia, Essential/etiology , Venous Thrombosis/diagnosis , Venous Thrombosis/therapyABSTRACT
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.
Subject(s)
Budd-Chiari Syndrome/physiopathology , Polycythemia Vera/physiopathology , Primary Myelofibrosis/physiopathology , Thrombocythemia, Essential/physiopathology , Venous Thrombosis/physiopathology , Adult , Aged , Budd-Chiari Syndrome/etiology , Female , Humans , Male , Middle Aged , Polycythemia Vera/complications , Portal Vein/physiopathology , Primary Myelofibrosis/complications , Proportional Hazards Models , Recurrence , Retrospective Studies , Risk Factors , Thrombocythemia, Essential/complications , Venous Thrombosis/etiologyABSTRACT
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.