The 2020 revision of the guidelines for the management of myeloproliferative neoplasms

Abstract

In 2016, the World Health Organization revised the diagnostic criteria for myeloproliferative neoplasms (MPNs) based on the discovery of disease-driving genetic aberrations and extensive analysis of the clinical characteristics of patients with MPNs. Recent studies have suggested that additional somatic mutations have a clinical impact on the prognosis of patients harboring these genetic abnormalities. Treatment strategies have also advanced with the introduction of JAK inhibitors, one of which has been approved for the treatment of patients with myelofibrosis and those with hydroxyurea-resistant or intolerant polycythemia vera. Recently developed drugs aim to elicit hematologic responses, as well as symptomatic and molecular responses, and the response criteria were refined accordingly. Based on these changes, we have revised the guidelines and present the diagnosis, treatment, and risk stratification of MPNs encountered in Korea.

Keywords: Polycythemia vera; Practice guideline; Primary myelofibrosis; Thrombocythemia, essential.

Figure 1

Recommended algorithm for the treatment of primary myelofibrosis. IPSS, International Prognostic Scoring System; DIPSS, Dynamic International Prognostic Scoring System. ^aRuxolitinib for low or intermediate-1 risk patients is approved by the Ministry of Food and Drug Safety but not currently covered by National Health Insurance system of Korea, ^bRuxolitinib treatment before transplantation to alleviate symptoms and splenomegaly can be considered.