Splanchnic vein thrombosis in myeloproliferative neoplasms

Sekhar, Mallika; McVinnie, Kathryn; Burroughs, Andrew K.

doi:10.1111/bjh.12461

Cited by 65 publications

(53 citation statements)

References 146 publications

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“…The persistent increased risk of liver cancer is likely related to underlying diseases such as liver cirrhosis, 20 whereas the increased risk of myeloproliferative neoplasms beyond 1 year of follow-up may indicate that diagnosis of these neoplasms was delayed. 21 We had no information on test results for the JAK2V617F mutation, but it is possible that the finding of this mutation was related to diagnosis of myeloproliferative neoplasms in some patients. 10 Alcohol abuse is a risk factor for SVT, but is also associated with smoking.…”

Section: Discussionmentioning

confidence: 99%

Splanchnic venous thrombosis is a marker of cancer and a prognostic factor for cancer survival

Søgaard

Farkas

Pedersen

et al. 2015

Blood

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Key Points• SVT is a marker of occult cancer, in particular myeloproliferative neoplasms, liver cancer, and pancreatic cancer.• SVT is a prognostic factor for short-term survival in patients diagnosed with liver or pancreatic cancer.It is unknown if splanchnic venous thrombosis (SVT) is a marker of occult cancer and a prognostic factor for cancer survival. Using Danish medical registries, we conducted a nationwide cohort study including all patients with first-time SVT (n 5 1191) between 1994 and 2011. We followed the patients for subsequent cancer diagnoses and calculated absolute risks and standardized incidence ratios (SIRs). We formed a matched comparison cohort of cancer patients without SVT, and assessed the prognostic impact of SVT on cancer survival by applying the Kaplan-Meier method and Cox regression. We followed the patients for a median of 1.6 years, and found that SVT was a marker of occult cancer. The 3-month cancer risk was 8.0% and the SIR was 33 (95% confidence interval, 27-40), compared with the general population. Increased risk was mainly found for liver cancer (risk 5 3.5%; SIR 5 1805), pancreatic cancer (risk 5 1.5%; SIR 5 256), and myeloproliferative neoplasms (risk 5 0.7%; SIR 5 764). The overall SIR remained increased twofold after 1 or more years of follow-up. SVT was also a prognostic factor for survival in patients with liver and pancreatic cancer. The clinical impact may be a more thorough diagnostic work-up in patients presenting with SVT. (Blood. 2015;126(8):957-963)

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Section: Discussionmentioning

confidence: 99%

Splanchnic venous thrombosis is a marker of cancer and a prognostic factor for cancer survival

Søgaard

Farkas

Pedersen

et al. 2015

Blood

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“…The management of PV-associated SVT requires continuing collaboration with hepatologists. 82 Cerebral vein thrombosis is a rare, life-threatening event that leads to a concurrent diagnosis of a MPN in 2% to 7% of cases; the estimated prevalence among all MPN is 1% to 2%. [83][84][85] A slightly higher frequency of cerebral vein thrombosis has been reported in PV than ET.…”

Section: Special Situations Of Interest Thrombosis In Unusual Sitesmentioning

confidence: 99%

How I treat polycythemia vera

Vannucchi

2014

Blood

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Polycythemia vera (PV) is a chronic myeloproliferative neoplasm associated with JAK2 mutations (V617F or exon 12) in almost all cases. The World Health Organization has defined the criteria for diagnosis, but it is still unclear which parameter (hemoglobin or hematocrit) is the most reliable for demonstrating increased red cell volume and for monitoring response to therapy; also, the role of bone marrow biopsy is being revisited. PV is associated with reduced survival because of cardiovascular complications and progression to post-PV myelofibrosis or leukemia. Criteria for risk-adapted treatment rely on the likelihood of thrombosis. Controlled trials have demonstrated that incidence of cardiovascular events is reduced by sustained control of hematocrit with phlebotomies (low-risk patients) and/or cytotoxic agents (high-risk patients) and antiplatelet therapy with aspirin. Hydroxyurea and interferon may be used as first-line treatments, whereas busulfan is reserved for patients that are refractory or resistant to first-line agents. However, there is no evidence that therapy improves survival, and the significance of reduction of JAK2 mutated allele burden produced by interferon is unknown. PV is also associated with a plethora of symptoms that are poorly controlled by conventional therapy. This article summarizes my approach to the management of PV in daily clinical practice.

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“…The presence of the JAK2 V617F mutations is diagnostic of MPN and accounts for 50% of patients with BCS. JAK2V617F mutation is found in 97% of patients with polycythaemia Vera as well as in approximately 60% of essential thrombocytopenia and myelofibrosis [3][4][5]. Forty percent of cases with BCS are JAK2V617F positive, although 17.1% of these do not have haematological features of MPN as defined by World Health Organisation (WHO) diagnostic criteria [6,7].…”

Section: Discussionmentioning

confidence: 99%

Case Report: A Rare and Unusual Cause of Acute Liver Failure

Chen¹

2015

J Gastrointest Dig Syst

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Budd-Chiari syndrome (BCS) is a rare condition caused by hepatic outflow obstruction. Typical clinical features include right upper quadrant pain, ascites and hepatosplenomegaly. We report a case of a 26 year old female who presented with jaundice, worsening abdominal swelling and abdominal pain. She subsequently developed acute liver failure and spontaneous bacterial peritonitis (SBP) with significant renal impairment requiring organ support. Computed tomography (CT) scan showed congested liver and occlusion of three hepatic veins consistent with BuddChiari Syndrome. Transjugular intra hepatic portosystemic stent/shunt (TIPSS) was inserted which resulted in complete resolution liver and renal function. She was subsequently found to be positive for the JAK-2 V617F mutation suggesting an underlying myeloproliferative disorder. This is a rare case in which a patient developed spontaneous bacterial peritonitis (SBP) in the context of BCS. The patient had excellent outcome from TIPSS procedure, which rescued her from undergoing liver transplantation.

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Splanchnic vein thrombosis in myeloproliferative neoplasms

Cited by 65 publications

References 146 publications

Splanchnic venous thrombosis is a marker of cancer and a prognostic factor for cancer survival

Splanchnic venous thrombosis is a marker of cancer and a prognostic factor for cancer survival

How I treat polycythemia vera

Case Report: A Rare and Unusual Cause of Acute Liver Failure

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