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Abstract In a population of 42 Philadelphia negative myeloproliferative neoplasm patients, all on systemic active treatment, the likelihood of responding to anti-SARS-CoV-2 BNT162b2 vaccine at 2 weeks after the second dose was significantly lower in the ten patients with myelofibrosis compared to the 32 with essential thrombocythemia (n = 17) and polycythemia vera (n = 15) grouped together, both in terms of neutralizing anti-SARS-CoV-2 IgG titers and seroprotection rates (32.47 AU/mL vs 217.97 AU/mL, p = 0.003 and 60% vs 93.8%, p = 0.021, respectively). Ruxolitinib, which was the ongoing treatment in five patients with myelofibrosis and three with polycythemia vera, may be implicated in reducing vaccine immunogenicity (p = 0.076), though large prospective study is needed to address this issue.

In a population of 42 Philadelphia negative myeloproliferative neoplasm patients, all on systemic active treatment, the likelihood of responding to anti-SARS-CoV-2 BNT162b2 vaccine at 2 weeks after the second dose was significantly lower in the ten patients with myelofibrosis compared to the 32 with essential thrombocythemia (n = 17) and polycythemia vera (n = 15) grouped together, both in terms of neutralizing anti-SARS-CoV-2 IgG titers and seroprotection rates (32.47 AU/mL vs 217.97 AU/mL, p = 0.003 and 60% vs 93.8%, p = 0.021, respectively). Ruxolitinib, which was the ongoing treatment in five patients with myelofibrosis and three with polycythemia vera, may be implicated in reducing vaccine immunogenicity (p = 0.076), though large prospective study is needed to address this issue. [ABSTRACT FROM AUTHOR]

Ph (Philadelphia chromosome) negative myeloproliferative neoplasms are chronic clonal disorders of haematopoietic stem cells, characterized by increased autonomic proliferation of one or more myeloid cell lines. They are a common prothrombogenic state found in patients with splanchnic vein thrombosis. The prevalence of the Ph negative myeloproliferative neoplasms is 40% in patients with Budd-Chiari syndrome and 30% in patients with portal vein thrombosis. The risk factors for splanchnic vein thrombosis are younger age, female sex, JAK2 V617F mutation, and concomitant hypercoagulable disorders. It appears that local endothelial changes play a significant role in the pathophysiology of splanchnic vein thrombosis. The treatment of splanchnic vein thrombosis is problematic, despite the anticoagulation treatment, the risk of recurrence after the thrombosis remains high. Therefore, it is necessary to verify the effectiveness of new treatment modalities in secondary prophylaxis. Moreover, the results from randomized controlled trials to support our treatment decisions are missing in these patients. The purpose of this article is to review the pathophysiology, clinical and molecular risk factors, and provides a practical guidance for the select an effective treatment strategy of splanchnic vein thrombosis in patients with Ph-negative myeloproliferative neoplasms. [ABSTRACT FROM AUTHOR]