For Publishers
DiscoveryMetadataPeer reviewHostingPublishing
For Researchers
JoinPublishReviewCollect
Register
Blog
About
Search
Advanced search
My ScienceOpen
Search
For Publishers
For Researchers
Blog
About
6
views
6
references
 Top references
cited by
0
    
0 reviews
 Review
0
comments
 Comment
0
recommends
+1 Recommend
0 collections
    0
    shares
      128
      similar
       All similar
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Pseudocirrhosis in Chronic Budd Chiari Syndrome With Janus Tyrosine Kinase 2 (JAK2) Mutation

      case-report
      Author(s): 1 , , 2 , 3 , 4
      Editor(s): ,
      Publication date (Electronic, pub):
      Publication date (Electronic, collection):
      Journal: Cureus
      Publisher: Cureus
      Keywords: cirrhosis, jak2 mutation, budd-chiari syndrome

      Read this article at

      ScienceOpenPublisherPMC
      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Budd-Chiari syndrome (BCS) occurs when there is hepatic venous outflow obstruction. Chronic BCS may result in liver cirrhosis due to long-standing obstruction and tend to present late. We present the first case of BCS secondary to Janus tyrosine kinase 2 (JAK2) mutation resulting in "pseudocirrhosis" rather than cirrhosis of the liver. Pseudocirrhosis clinically and radiologically mimics cirrhosis without the classical histopathological changes, and it is usually associated with metastatic cancers.

          Related collections

          Most cited references6

          • Record: found
          • Abstract: found
          • Article: not found

          Incidence of the JAK2 V617F mutation among patients with splanchnic or cerebral venous thrombosis and without overt chronic myeloproliferative disorders.

          E. Rossi, G Leone, P. Chiusolo … (2007)
          Thrombosis of splanchnic or cerebral veins is a typical manifestation of polycythemia vera (PV) or essential thrombocythemia (ET). The recently identified Janus kinase 2 (JAK2) V617F somatic mutation is closely related to chronic myeloproliferative disorders (CMD). To assess the incidence of the JAK2 V617F mutation among patients with splanchnic or cerebral venous thrombosis with or without overt CMD. We searched for the mutation in 139 adult patients (> 18 years old) with thrombosis of hepatic veins (HVT, n = 15), or extrahepatic portal vein (PVT) and/or mesenteric vein (MVT) (n = 79), or cerebral veins (CVT, n = 45). Only 19 patients fulfilled criteria for diagnosis of PV (n = 8) or ET (n = 11) at the time of thrombosis: four had HVT, 11 PVT and/or MVT, and four CVT. The JAK2 V617F mutation was found in 94.7% [95% CI 75.3-99.0] of the patients with overt CMD at the time of thrombosis, in 21.5% (95% CI 13.8-31.7) of the patients with abdominal venous thrombosis and without overt CMD, and in 4.8% (95% CI 1.3-16.1) of the patients with CVT and without overt CMD. Among the patients without overt CMD or thrombophilia and with unprovoked thrombosis, 29.4% (95% CI 16.8-46.1) with splanchnic venous thrombosis and 42.8% (95% CI 24.4-63.4) with PVT had the JAK2 V617F mutation. A substantial proportion of patients with splanchnic venous thrombosis and a small, but significant, number of patients with CVT can be recognized as carriers of the JAK2 V617F mutation in the absence of overt signs of CMD. The clinical significance of such findings deserves further investigation.
          Article 0 comments Cited 28 times – based on 0 reviews     Review now
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            The changing scene of hepatic vein thrombosis: recognition of asymptomatic cases.

            Antoine Hadengue, Marc Poliquin, Valérie Vilgrain … (1994)
            Hepatic vein thrombosis is thought to be manifested by ascites, abdominal pain, and hepatomegaly, with a uniformly poor prognosis. However, new imaging techniques allow for the diagnosis of hepatic vein thrombosis in asymptomatic cases. The aim of our study was to re-evaluate symptoms and prognosis in patients with hepatic vein thrombosis. Eighty-one patients with hepatic vein thrombosis were analyzed. Forty-seven patients were admitted from 1970 to June 1987 (group I, before Doppler ultrasonography and magnetic resonance imaging were introduced at our hospital) and 34 from July 1987 to June 1991 (group II). When comparing the two groups, age, sex ratio, and causes of hepatic vein thrombosis did not differ. Eight group II patients (asymptomatic patients) had no ascites, hepatomegaly, or abdominal pain. One major hepatic vein remained patent in 41% of group II patients, compared with 12% in group I (P < 0.05). Intrahepatic collaterals were seen in 79% of group II patients, compared with 21% of group I patients (P < 0.01). All asymptomatic patients had large intrahepatic and portasystemic collaterals. At 3 years, death occurred in 22% of group II patients and in 45% of group I patients. No asymptomatic patient died. Asymptomatic hepatic vein thrombosis is associated with the spontaneous development of large intrahepatic and portosystemic collaterals. In asymptomatic patients, prognosis at 3 years seems to be good, and surgical therapy may not be required.
            Article 0 comments Cited 27 times – based on 0 reviews     Review now
              Bookmark
              • Record: found
              • Abstract: found
              • Article: found
              Is Open Access

              Pseudocirrhosis: A Case Series and Literature Review

              Abimbola Adike, Nina Karlin, Christine O Menias … (2016)
              Pseudocirrhosis describes morphological changes of the liver that closely mimic cirrhosis, without the typical histopathological changes seen in cirrhosis. It most commonly occurs in patients with metastatic breast cancer, although it has been reported in other malignancies as well. Like in cirrhosis, portal hypertension is often seen in patients with pseudocirrhosis. Pseudocirrhosis is a rare but important complication of metastatic cancer. In this case series and literature review, we describe 6 patients with hormone-receptor-positive metastatic breast cancer. We report the significant morbidity associated with pseudocirrhosis in the course of treatment in patients with metastatic breast cancer.
              Article 0 comments Cited 18 times – based on 0 reviews     Review now
                Bookmark
                All references

                Author and article information

                Journal
                Journal ID (nlm-ta): Cureus
                Journal ID (iso-abbrev): Cureus
                Journal ID (issn): 2168-8184
                Title: Cureus
                Publisher: Cureus (Palo Alto (CA) )
                ISSN (Electronic): 2168-8184
                Publication date (Electronic, pub): 23 July 2020
                Publication date (Electronic, collection): July 2020
                Volume: 12
                Issue: 7
                Electronic Location Identifier: e9355
                Affiliations
                [1 ] Internal Medicine, Roger Williams Medical Center, Boston University School of Medicine, Providence, USA
                [2 ] Internal Medicine, Geriatric Research, Education and Clinical Center, Bruce W. Carter Veterans Affairs Medical Center, Miami, USA
                [3 ] Internal Medicine, Roger Williams Medical Center, Providence, USA
                [4 ] Gastroenterology, Roger Williams Medical Center, Providence, USA
                Author notes
                Article
                DOI: 10.7759/cureus.9355
                PMC ID: 7444998
                PubMed ID: 32850227
                SO-VID: 40082422-9191-4128-9a80-730d4809f94c
                Copyright © Copyright © 2020, Lakshmanan et al.
                License:

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                Date received : 8 July 2020
                Date accepted : 23 July 2020
                Categories
                Subject: Internal Medicine
                Subject: Gastroenterology
                Subject: Hematology

                Keywords: cirrhosis,jak2 mutation,budd-chiari syndrome
                Data availability:
                Keywords: cirrhosis, jak2 mutation, budd-chiari syndrome

                Comments

                Comment on this article