A guideline for the diagnosis and management of polycythaemia vera. A British Society for Haematology Guideline

McMullin, Mary Frances; Cn, Harrison; Ali, Simak; Cargo, Catherine; Chen, Frederick; Ewing, Joanne; Garg, Mamta; Godfrey, Anna L.; Sn, Singh; McLornan, Donal P.; Nangalia, Jyoti; Sekhar, Mallika; Wadelin, Frances; Aj, Mead

doi:10.1111/bjh.15648

Cited by 113 publications

(182 citation statements)

References 96 publications

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“…In recent years, a number of national and international guidelines for the diagnosis and treatment of patients with MPNs have been published or updated to reflect advances in the understanding of the disease and the availability of new treatment options (Table 1) [27][28][29][30][31][32][33][34]. The discovery of the three driver mutations has also led to mutation screening currently being recommended as part of the diagnostic criteria of MPNs [27][28][29][30][31][32]34]. The guidelines provide similar advice with regard to treatment, although, in some cases, they reflect availability and payment status of particular drugs, or local practice.…”

Section: Treating Mpnsmentioning

confidence: 99%

The Myeloproliferative Neoplasm Landscape: A Patient’s Eye View

Petruk¹,

Mathias²

2020

Adv Ther

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Section: Treating Mpnsmentioning

confidence: 99%

The Myeloproliferative Neoplasm Landscape: A Patient’s Eye View

Petruk¹,

Mathias²

2020

Adv Ther

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“…11 A relationship between platelet count (either at diagnosis or follow-up) and thrombotic risk has not been established in PV, but severe thrombocytosis (!1,500 Â 10 9 /L) is associated with increased risk of bleeding due to acquired von Willebrand syndrome (AVWS) and should be considered an indication for cytoreductive therapy. 1,11 A hallmark of patients presenting with MPN related thrombosis is presence of the JAK2 (V617F) mutation, which has a strong link with thrombosis in both venous and arterial systems. The JAK2 (V617F) mutation is present in 95% of patients with PV and around 60% patients with ET or PMF.…”

Section: Pathogenesis Of Thrombosis In Myeloproliferative Neoplasmsmentioning

confidence: 99%

“…Given the high mortality associated with thrombotic events in patients with PV, the first goal of therapy is to reduce the risk of thrombosis, which is achieved mainly by controlling Hct to < 0.45. 1 However, frequent venesection in PV patients should be done with caution as it can cause thrombocytosis secondary to iron deficiency. Combined cytoreduction and periodic venesection can be used overcome this complication.…”

Section: Primary Prophylaxis For Prevention Of Thrombosis In Myelopromentioning

confidence: 99%

Pathogenesis and Management of Thrombotic Disease in Myeloproliferative Neoplasms

Arachchillage

Laffan

2019

Semin Thromb Hemost

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Chronic myeloproliferative neoplasms (MPN) are characterized by clonal expansion of an abnormal hematopoietic stem/progenitor cell and include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). Venous thrombosis, often at unusual sites, including splanchnic vein thrombosis and arterial thrombosis, as well as a hemorrhagic tendency and a propensity to transform into myelofibrosis or acute leukemia are common complications in patients with MPNs. The pathogenesis of thrombosis in MPN patients is complex and multifactorial. Disease related factors, such as an increase in blood cell counts (i.e., leukocytosis, erythrocytosis, and thrombocytosis), and more importantly presence of JAK2 mutation can interact with non-disease patient related factors such as age, previous history of thrombotic events, obesity, hypertension, hyperlipidemia, and presence of thrombophilic defects. The overall rate of recurrent thrombosis after venous thromboembolism (VTE) is 6.0 to 6.5 per 100 patient-years in patients with MPN compared to 2.7 to 3.7 per 100 patient-years in patients without MPN, and antithrombotic therapy with vitamin K antagonists (VKAs) is associated with a clear benefit, reducing the incidence of recurrence by 48 to 69%. Life-long oral anticoagulation with VKAs is the cornerstone of the antithrombotic treatment for splanchnic vein thrombosis (SVT). Patients with MPN-related cerebral venous thrombosis (CVT) should also be treated with long-term anticoagulation with VKAs. The role of direct acting oral anticoagulants in patients with thrombosis and MPN is not established and the use of these anticoagulants should be considered on an individual basis according to the risk of recurrent of VTE and bleeding.

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“…It also assumes that it is possible to reliably discriminate between PV and ET on the basis of the morphological appearance of the bone marrow. The British Society for Haematology (BSH) have reviewed the evidence and suggested that criteria for the diagnosis of PV should be high HCT >0.52% (men), >0.48% (women) or a raised red cell mass (>25% above predicted) and a mutation in JAK2 with more detailed criteria for the very rare JAK2 mutation negative 12 . These criteria are likely to be more practical in defining those with PV without flagging too many of those with Hbs within the normal range as in need of investigation.…”

Section: Diagnostic Criteria For Polycythemia Vera In 2019mentioning

confidence: 99%

Diagnostic workflow for hereditary erythrocytosis and thrombocytosis

McMullin

2019

Hematology

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In the patient presenting with an elevated blood count who does not have an acquired clonal disorder causing a myeloproliferative neoplasm, hereditary erythrocytosis or hereditary thrombocytosis needs to be considered as a possible explanation. A young patient and/or those with a family history of myeloproliferative neoplasm should specifically raise this possibility. Among the causes of hereditary erythrocytosis are mutations in the genes in the oxygen sensing pathway and high-affinity hemoglobins. Hereditary thrombocytosis has been shown to be accounted for by mutations in THPO, MPL, and JAK2 genes. In those who have a possible hereditary erythrocytosis or thrombocytosis, the investigative pathway includes specific investigation to rule out the more common acquired clonal disorders, and, if indicated, other secondary causes, measurement of specific cytokines as indicated, and search for specific identified molecular lesions that have been shown to cause these hereditary disorders. There remain individuals who appear to have a hereditary disorder in whom a genetic lesion cannot currently be identified.

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A guideline for the diagnosis and management of polycythaemia vera. A British Society for Haematology Guideline

Cited by 113 publications

References 96 publications

The Myeloproliferative Neoplasm Landscape: A Patient’s Eye View

The Myeloproliferative Neoplasm Landscape: A Patient’s Eye View

Pathogenesis and Management of Thrombotic Disease in Myeloproliferative Neoplasms

Diagnostic workflow for hereditary erythrocytosis and thrombocytosis

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