Incidence of Silent Thrombosis in Patients Younger Than 60 Years With Myeloproliferative Neoplasms: Single-Center Egyptian Study

Clin Lymphoma Myeloma Leuk. 2019 Aug;19(8):e425-e429. doi: 10.1016/j.clml.2019.05.005. Epub 2019 May 13.

Abstract

Background: Identification of janus kinase 2 (JAK2) mutation even in absence of myeloproliferative disorders (MPNs) was found to be related to venous thromboembolism occurrence. Venous thrombosis screening is not routinely requested in patients with myeloproliferative neoplasms unless the patient is symptomatic. It has been reported that the incidence of thrombosis in elderly patients is much higher than in young patients. The aim of this work was to screen MPN patients for venous thrombosis and study its correlation with JAK2 allele burden and with MPN 10 score.

Patients and methods: We enrolled 73 patients with JAK2-positive MPN from our Hematology Clinic in the period August 2015 to Feb 2017. All patients had been screened for thrombosis in the venous system in lower limbs (LLs), upper limbs, portal, and mesenteric systems using color Doppler ultrasound imaging.

Results: Fifty-three (72.6%) patients were younger than 60 years. Twenty-two (30%) had essential thrombocytosis, 35 (47.9%) had polycythemia rubra vera, and 16 (22%) had idiopathic myelofibrosis. Twenty-seven venous thrombotic attacks were reported in 22 (30.1%) patients. Five (6.8%) had thrombosis in 2 sites. Seventeen (23%) had superior mesenteric and portal vein thrombosis. Six (8%) had iliofemoral (8%) and 4 (5%) had combined LL and portal thrombosis. Eight (10.8%) had active thrombosis at screening. Only 3 patients (4%) were symptomatic with abdominal pain during screening. Pruritis (P = .02) and abdominal pain (P = .039) were significantly different between cases with and without thrombosis. There was no significant difference in MPN 10 score between cases with active or previous thrombosis.

Conclusion: We recommend routine screening for venous thrombosis in any case of MPN when diagnosed and screening for MPNs in any patient with venous thrombosis especially of the portal vein or atypical sites. If MPN patients present with increasing pruritus or abdominal pain, they also should be screened for venous thrombosis. Further research on a large scale in MPN age groups younger than 60 years regarding pathogenesis of thrombosis is highly recommended.

Keywords: Doppler US; JAK2; MPN10; Myeloproliferative disorders; Venous thrombosis.

Publication types

  • Clinical Trial

MeSH terms

  • Adult
  • Aged
  • Alleles
  • Egypt / epidemiology
  • Female
  • Follow-Up Studies
  • Humans
  • Incidence
  • Janus Kinase 2 / genetics*
  • Male
  • Middle Aged
  • Mutation*
  • Myeloproliferative Disorders / complications*
  • Prognosis
  • Thrombosis / diagnosis
  • Thrombosis / epidemiology*
  • Thrombosis / etiology
  • Young Adult

Substances

  • JAK2 protein, human
  • Janus Kinase 2