A guideline for the management of specific situations in polycythaemia vera and secondary erythrocytosis

McMullin, Mary Frances; Mead, Adam J.; Ali, Sahra; Cargo, Catherine; Chen, Frederick; Ewing, Joanne; Garg, Mamta; Godfrey, Anna L.; Knapper, Steven; McLornan, Donal P.; Nangalia, Jyoti; Sekhar, Mallika; Wadelin, Frances; Harrison, Claire

doi:10.1111/bjh.15647

Cited by 88 publications

(110 citation statements)

References 103 publications

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“…In many centres, the indication for cytoreduction is personalised depending on individual circumstances. At the pathophysiological level it is increasingly clear that the thrombogenicity of MPN is due to qualitative as much as quantitative changes of blood cells …”

Section: Medical Treatmentmentioning

confidence: 99%

Review article: a multidisciplinary approach to the diagnosis and management of Budd‐Chiari syndrome

Khan

Armstrong

Mehrzad

et al. 2019

Aliment Pharmacol Ther

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Background: Budd-Chiari syndrome (BCS) is a rare but fatal disease caused by obstruction in the hepatic venous outflow tract. Aim: To provide an update of the pathophysiology, aetiology, diagnosis, management and follow-up of BCS. Methods: Analysis of recent literature by using Medline, PubMed and EMBASE databases. Results: Primary BCS is usually caused by thrombosis and is further classified into "classical BCS" type where obstruction occurs within the hepatic vein and "hepatic vena cava BCS" which involves thrombosis of the intra/suprahepatic portion of the inferior vena cava (IVC). BCS patients often have a combination of prothrombotic risk factors. Aetiology and presentation differ between Western and certain Asian countries. Myeloproliferative neoplasms are present in 35%-50% of European patients and are usually associated with the JAK2-V617F mutation. Clinical presentation is diverse and BCS should be excluded in any patient with acute or chronic liver disease. Non-invasive imaging (Doppler ultrasound, computed tomography, or magnetic resonance imaging) usually provides the diagnosis. Liver biopsy should be obtained if small vessel BCS is suspected. Stepwise management strategy includes anticoagulation, treatment of identified prothrombotic risk factors, percutaneous revascularisation and transjugular intrahepatic portosystemic stent shunt to re-establish hepatic venous drainage, and liver transplantation in unresponsive patients. This strategy provides a 5-year survival rate of nearly 90%. Long-term outcome is influenced by any underlying haematological condition and development of hepatocellular carcinoma. Conclusions: With the advent of newer treatment strategies and improved understanding of BCS, outcomes in this rare disease have improved over the last three decades. An underlying haematological disorder can be the major determinant of outcome. Primary BCS is considered a multifactorial disease and multicentre data found a combination of several prothrombotic conditions in 25% to 46% of the patients with BCS, 11,19-21 several times greater than expected in the general population. The discovery of one causal factor should not discourage further investigation to identify other prothrombotic conditions. Multifactorial causes may explain the rarity of BCS. 2 The prothrombotic conditions found in BCS, in particular the classical type, include: Factor V Leiden mutation, prothrombin (PT) gene mutation, protein C deficiency, protein S deficiency, antithrombin deficiency, antiphospholipid syndrome, hyperhomocysteinemia and paroxysmal nocturnal haemoglobinuria. 22 BCS is also associated with systemic inflammatory diseases, such as Behçet's disease, sarcoidosis, vasculitis and other connective tissue diseases. 22 A detailed description of the frequency of inherited/acquired thrombophilias and risk factors found in patients with BCS compared to those with portal vein thrombosis is summarised by Poisson and colleagues. 23

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Section: Medical Treatmentmentioning

confidence: 99%

Review article: a multidisciplinary approach to the diagnosis and management of Budd‐Chiari syndrome

Khan

Armstrong

Mehrzad

et al. 2019

Aliment Pharmacol Ther

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“…However, routine testing for inherited or acquired thrombophilia is not recommended in patients with MPN since this will not change the management. 10 In the ECLAP study, age !65 years and a prior history of thrombosis were found to be the most important predictors of cardiovascular events. 3 These factors have consistently proven to be independent predictors of future events in PV, ET, and PMF.…”

Section: Pathogenesis Of Thrombosis In Myeloproliferative Neoplasmsmentioning

confidence: 99%

“…Abnormalities in red blood cells, including biochemical changes in the cell membrane and content, may independently impair blood flow as well, through the formation of red cell aggregates. 10 Furthermore, red cell aggregation facilitates platelet and leukocyte interaction with the vessel wall. ►Fig.…”

Section: Pathogenesis Of Thrombosis In Myeloproliferative Neoplasmsmentioning

confidence: 99%

“…18 Patients with secondary erythrocytosis who are symptomatic because of hyperviscosity or have a Hct > 0.56 should be considered for venesection to reduce this to 0.50 to 0.52. 10 The risk for thrombosis in patients with JAK2 V617F mutation is at least twice higher compared to patients with CALR mutation. 19 Differences in the thrombotic risk in patients with CALR mutations and JAK2 mutated patients are most likely explained by the fact that patients with CALR mutation have been reported to have an earlier disease 10 onset, higher platelet count but lower WBC count and hemoglobin level compared with those with JAK2 V617F.…”

Section: Pathogenesis Of Thrombosis In Myeloproliferative Neoplasmsmentioning

confidence: 99%

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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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“…In our case, the risk of haemorrhage was expected to be largely due to surgical factors as our patient lacked thrombocytosis and had no previous bleeding episodes. Tranexamic acid has been suggested as a potential treatment option for bleeding in polycythaemia vera from evidence in von Willebrand disease, but specific trials in polycythaemia vera are lacking and it was not used in our case .…”

Section: Discussionmentioning

confidence: 99%

Laparoscopic hepatectomy in a patient with uncontrolled polycythaemia vera

Riekki

Ling

Cordovani

2019

Anaesthesia Reports

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This case report describes the peri-operative course of a patient with uncontrolled polycythaemia vera who underwent a laparoscopic hepatectomy for intrahepatic cholangiocarcinoma. Polycythaemia vera is a chronic condition that results in erythrocytosis and puts patients at risk of peri-operative complications including thrombotic events and paradoxical haemorrhage. Little evidence exists on the ideal peri-operative management of uncontrolled polycythaemia vera when the proposed procedure carries a high risk of haemorrhage. Our patient presented with a pre-operative haemoglobin of 197 g.l À1 (haematocrit 65%) and was not phlebotomised pre-operatively. Intra-operatively he lost 2700 ml of blood, reducing his haematocrit to 48%, and then suffered fatal thrombotic complications postoperatively. The patient did not receive any blood product transfusions during his peri-operative course. We review the available evidence to guide the perioperative management of patients with polycythaemia vera. The inherent risks of thrombosis and haemorrhage associated with polycythaemia vera need to be weighed against the specific surgical and transfusion-related risks. Phlebotomy to achieve a pre-operative haematocrit under 45% is recommended and intra-operative phlebotomy shows promise for reducing blood loss during hepatectomies. Management of postoperative erythrocytosis may be an important and underappreciated aspect of reducing the peri-operative risk of thrombosis in patients with polycythaemia vera.

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A guideline for the management of specific situations in polycythaemia vera and secondary erythrocytosis

Cited by 88 publications

References 103 publications

Review article: a multidisciplinary approach to the diagnosis and management of Budd‐Chiari syndrome

Review article: a multidisciplinary approach to the diagnosis and management of Budd‐Chiari syndrome

Pathogenesis and Management of Thrombotic Disease in Myeloproliferative Neoplasms

Laparoscopic hepatectomy in a patient with uncontrolled polycythaemia vera

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