[Splanchnic vein thrombosis as a first manifestation of Primary myelofibrosis]

Gac Med Mex. 2017;153(4):537-540. doi: 10.24875/GMM.17002822.
[Article in Spanish]

Abstract

Myeloproliferative neoplasms are chronic disorders of clonal hematopoietic stem cells, characterized by an overproduction of functional granulocytes, red blood cells and / or platelets, and one of the major complications is the occurrence of venous and arterial thrombotic problems caused by increased platelet aggregation and thrombin generation. In this study 11 cases of primary myelofibrosis (PM) were evaluated and 2 debuted with splanchnic venous thrombosis (SVT); so after seeing the results of this study and of world literature, it is suggested that in patients with SVT, diagnostic methods for PM like the JAK2V617F mutation should be included.

Las neoplasias mieloproliferativas (NMP) son alteraciones crónicas de las células madre hematopoyéticas clonales caracterizadas por una mayor producción de granulocitos, glóbulos rojos o plaquetas. Una de las principales complicaciones de las NMP es la aparición de problemas trombóticos venosos y arteriales causados por un aumento en la agregación plaquetaria y la generación de trombina. Se evaluaron 11 casos de mielofibrosis primaria (MP), de los cuales dos debutaron con trombosis venosa esplácnica (TVE). Tras observar los resultados de este estudio y de la literatura mundial, se sugiere que al evaluar pacientes con TVE se incluyan métodos diagnósticos para MP, como la mutación JAK2V617F.

Keywords: JAK2V617F mutation; Mielofibrosis primaria; Mutación JAK2V617F; Myeloproliferative neoplasms; Neoplasias mieloproliferativas; Primary myelofibrosis; Splanchnic vein thrombosis; Trombosis venosa esplácnica.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Female
  • Humans
  • Janus Kinase 2 / genetics*
  • Male
  • Middle Aged
  • Mutation
  • Primary Myelofibrosis / complications
  • Primary Myelofibrosis / diagnosis*
  • Primary Myelofibrosis / genetics
  • Splanchnic Circulation
  • Venous Thrombosis / etiology*

Substances

  • JAK2 protein, human
  • Janus Kinase 2