Zuzana Jedináková, Juraj Chudej, Tomáš Balhárek, Juraj Marcinek, Ján Staško
Myeloproliferative neoplasms (MPN) affect typically the patients in older age. They are characterised by increased occurrence
of thrombotic complications, which affect the patients‘ prognosis and quality of life. Arterial thrombosis dominates
in older age, venous thrombotic events occur less frequently. Thrombotic complications in atypical localisation,
predominantly in splanchnic region, are associated with high probability of MPN. We present the rare case of 23-years
old man with myeloproliferative disease with manifestation in childhood – permanent mild thrombocytosis, from 16
years of age known coagulopathy with deficiency of coagulation factors. Despite the long-lasting follow up by paediatrician
due to mild thrombocytosis, diagnose of primary myelofibrosis was established at the stage of severe portal hypertension
and massive spleen enlargement with cavernous transformation of portal vein.