[Investigation and treatment of prefibrotic/early primary myelofibrosis. A case study].

Orv Hetil

II. Belgyógyászati Klinika, Belgyógyászati Intézet, Hematológiai Tanszék, Debreceni Egyetem, Általános Orvostudományi Kar Debrecen, Nagyerdei krt. 98., 4032.

Published: April 2018

AI Article Synopsis

  • - A 47-year-old woman with a history of various health issues presented with elevated platelet counts (617 G/L) due to persistent thrombocytosis, leading to further examination for potential underlying diseases.
  • - Imaging revealed a 4.5 cm thrombus in her left renal artery, prompting the start of a heparin treatment regimen; genetic testing confirmed the presence of the JAK2V617F mutation.
  • - A bone marrow analysis indicated early-stage myelofibrosis, resulting in treatment with hydroxyurea, which ultimately relieved her symptoms and helped normalize her platelet count.

Article 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. Orv Hetil. 2018; 159(15): 603-609.

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Source
http://dx.doi.org/10.1556/650.2018.30995DOI Listing

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