A korai/praefibroticus primer myelofibrosis kivizsgálása és kezelése egy eset kapcsán

Translated title of the contribution: Investigation and treatment of prefibrotic/early primary myelofibrosis

Magyari Ferenc, Bedekovics Judit, Décsy Judit, Ilonczai Péter, A. Illés, Simon Zsófia

Research output: Contribution to journalArticle

Abstract

Moderate thrombocytosis can accompany several diseases (bleeding, inflammation, iron deficiency, or autoimmune diseases), but hematologic examination is strongly recommended in a patient with persistent platelet count above 450 G/L unless reactive origin can be confirmed. The 47-year-old woman's medical history included hypertonia, asthma bronchiale, and endometriosis. In March 2015, she underwent laboratory examination due to weight loss and lack of appetite. Her results showed elevated thrombocyte count (617 G/L), but no iron deficiency. She presented in our clinic on 07. 04. 2015 with acute pain below her left hypochondrial region, but simple imaging examinations showed no difference to explain it. Abdominal CT revealed a 4.5 cm thrombus which protruded into the left renal artery, blocking it. We started APTI- (activated partial thromboplastin time) monitored continuous intravenous treatment with unfractionated heparin. The JAK2V617F mutation analysis came back positive. Subsequent bone marrow examination revealed prefibrotic/early stage myelofibrosis, prompting treatment with hydroxyurea. The applied treatments led to the disappearance of the patient's symptoms accompanied by the gradual normalisation of the thrombocyte count. Moderate thrombocytosis is often secondary, but if it persists and is accompanied by mainly thromboembolic events, the risk of diseases of the haematopoietic system, primarily Philadelphia chromosome negative chronic myeloproliferative disease should also be considered. Clinically, essential thrombocythaemia and the prefibrotic/early stage of myelofibrosis can be very similar. Differential diagnosis is only possible through the histological examination of the bone marrow, which becomes indispensible due to the difference in prognosis and treatment options.

Translated title of the contributionInvestigation and treatment of prefibrotic/early primary myelofibrosis
Original languageHungarian
Pages (from-to)603-609
Number of pages7
JournalOrvosi Hetilap
Volume159
Issue number15
DOIs
Publication statusPublished - Apr 1 2018

ASJC Scopus subject areas

  • Medicine(all)

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