Annals of Hematology

, Volume 86, Issue 11, pp 793–800

JAK2V617F positive early stage myeloproliferative disease (essential thrombocythemia) as the cause of portal vein thrombosis in two middle-aged women: therapeutic implications in view of the literature

  • J. J. Michiels
  • S. Commandeur
  • G. J. Hoogenboom
  • J. J. Wegman
  • L. Scholten
  • R. H. van Rijssel
  • H. De Raeve
Original Article

DOI: 10.1007/s00277-007-0351-1

Cite this article as:
Michiels, J.J., Commandeur, S., Hoogenboom, G.J. et al. Ann Hematol (2007) 86: 793. doi:10.1007/s00277-007-0351-1

Abstract

The present study describes portal vein thrombosis (PVT) in two women as the first and single presenting symptom of latent or masked myeloproliferative disease (MPD). Essential thrombocythemia (ET) was suspected by a sustained increase in platelet count (>400 × 109/l) and slight splenomegaly on echogram. ET could be diagnosed by the presence of large platelet in peripheral blood smear, an increase in clustered large megakaryocytes in bone marrow smear and the presence of the JAK2V617F mutation. A subsequent biopsy specimen was consistent with the diagnosis of true ET. In patients with a first episode of splanchnic vein thrombosis (SVT), analysis of any venous thrombophilic risk factors as well as a JAK2V617F mutation status indicative for MPD is warranted. Administration of heparin followed by oral anticoagulation with vitamin K antagonists is the treatment of choice in patients with SVT. Anticoagulation therapy combined with low-dose aspirin and proper treatment of the MPD is recommended in patients with SVT associated with the JAK2V617F mutation.

Keywords

Budd–Chiari syndrome (BCS)Splanchnic vein thrombosis (SVT)Hepatic vein thrombosis (HVT)Portal vein thrombosis (PVT)ThrombophiliaMyeloproliferative disorders (MPD)JAK2V617F mutation

Copyright information

© Springer-Verlag 2007

Authors and Affiliations

  • J. J. Michiels
    • 1
    • 7
  • S. Commandeur
    • 2
  • G. J. Hoogenboom
    • 3
  • J. J. Wegman
    • 3
  • L. Scholten
    • 4
  • R. H. van Rijssel
    • 5
  • H. De Raeve
    • 6
  1. 1.Department of HematologyUniversity Hospital AntwerpEdegem, AntwerpBelgium
  2. 2.Department of DermatologyLeiden University Medical CenterLeidenThe Netherlands
  3. 3.Department of Internal MedicineCZ Deventer HospitalDeventerThe Netherlands
  4. 4.Clinical ChemistryCZ Deventer HospitalDeventerThe Netherlands
  5. 5.Department of PathologyDeventer HospitalDeventerThe Netherlands
  6. 6.Department of PathologyUniversity Hospital AntwerpAntwerpBelgium
  7. 7.European Working Group on Myeloproliferative Disorders (EWG.MPD)Goodheart InstituteRotterdamThe Netherlands