Philadelphia-Negative Chronic Myeloproliferative Neoplasms during the COVID-19 Pandemic: Challenges and Future Scenarios

Abstract

An outbreak of severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) started in December 2019 in China and then become pandemic in February 2020. Several publications investigated the possible increased rate of COVID-19 infection in hematological malignancies. Based on the published data, strategies for the management of chronic Philadelphia-negative chronic myeloproliferative neoplasms (MPNs) are provided. The risk of severe COVID-19 seems high in MPN, particularly in patients with essential thrombocythemia, but not negligible in myelofibrosis. MPN patients are at high risk of both thrombotic and hemorrhagic complications and this must be accounted in the case of COVID-19 deciding on a case-by-case basis. There are currently no data to suggest that hydroxyurea or interferon may influence the risk or severity of COVID-19 infection. Conversely, while the immunosuppressive activity of ruxolitinib might pose increased risk of infection, its abrupt discontinuation during COVID-19 syndrome is associated with worse outcome. All MPN patients should receive vaccine against COVID-19; reassuring data are available on efficacy of mRNA vaccines in MPNs.

Covid-19 in Philadelphia-negative myeloproliferative neoplasms: a GIMEMA survey on incidence, clinical management and vaccine

“…During the first two years of the coronavirus disease 2019 (Covid-19) pandemic caused by the spreading of the coronavirus SARS-CoV-2, several publications investigated about the incidence of Covid-19 infection in Philadelphia-negative chronic myeloproliferative neoplasms (MPN), showing an increased risk of thromboembolism in essential thrombocythemia and of mortality in myelofibrosis [ 1 ]. In many studies, the risk of SARS-CoV2 infection seemed not elevated [ 2 , 3 ]; in a large study by the Italian Hematology Alliance, out of 536 patients included, 15% of them were affected by MPN [ 4 ].…”

“…After the vaccination, among the MPN patients tested for anti-spike antibodies, it seems that a seroconversion was achieved in all ET patients, in more than 80% of PV patients, while 72.9% of MF patients achieved a response. It has been reported that MPN patients may have lower response to vaccination due to immunological competence [ 1 , 8 , 9 ]. As suggested by other reports, the results of the survey confirmed that among MPN a reduced seroconversion should be considered in MF: the negative role of ruxolitinib for the possible seroconversion has been reported in different small cohorts of patients [ 10 – 13 ], suggesting the need for a full vaccination course.…”

Analysis of the Clinical Significance and Safety of Interferon in the Treatment of Chronic Myeloproliferative Tumors

“…Zhang et al [ 15 ] found that IFN- α specifically blocked the proliferation advantage of JAK2V617F mutant hematopoietic stem cells in mice, thereby preventing the development of MPN and even achieving eradication [ 16 ]. Clinical research has reported that some patients with MPN treated with IFN achieved complete hematologic and molecular biological remission [ 17 ]. However, the clinical efficacy of IFN on JAK2V617F mutation-positive patients has been marginally explored [ 18 ].…”

Clinical Course and Prophylaxis of COVID-19 in Patients with Myeloproliferative Diseases

“…Было зарегистрировано всего 2 случая тромбоза (у пациентов с эссенциальной тромбоцитемией), что может быть связано с особенностью дизайна исследования и проводимой антитромботической профилактикой. [5]. Ввиду особенностей патогенеза данных заболеваний пациенты с МПЗ подвержены высокому риску как тромботических, так и геморрагических осложнений, что необходимо учитывать при принятии решений по тактике ведения COVID-19 в каждом конкретном случае [5][6][7].…”

“…[5]. Ввиду особенностей патогенеза данных заболеваний пациенты с МПЗ подвержены высокому риску как тромботических, так и геморрагических осложнений, что необходимо учитывать при принятии решений по тактике ведения COVID-19 в каждом конкретном случае [5][6][7]. При этом МПЗ и COVID-19 имеют общие патогенетические пути гиперкоагуляции и тромбоза.…”