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Aroldi, Andrea; Cecchetti, Caterina; Colombo, Arianna; Cattaneo, Leonardo; Pioltelli, Pietro Enrico; Pogliani, Enrico Maria; Elli, Elena Maria

Neurological symptoms in essential thrombocythemia: impact of JAK2V617F mutation and response to therapy

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  • Medientyp: E-Artikel
  • Titel: Neurological symptoms in essential thrombocythemia: impact of JAK2V617F mutation and response to therapy
  • Beteiligte: Aroldi, Andrea; Cecchetti, Caterina; Colombo, Arianna; Cattaneo, Leonardo; Pioltelli, Pietro Enrico; Pogliani, Enrico Maria; Elli, Elena Maria
  • Erschienen: Wiley, 2016
  • Erschienen in: European Journal of Haematology
  • Sprache: Englisch
  • DOI: 10.1111/ejh.12638
  • ISSN: 0902-4441; 1600-0609
  • Entstehung:
  • Anmerkungen:
  • Beschreibung: <jats:title>Abstract</jats:title><jats:p>Patients with essential thrombocythemia (<jats:styled-content style="fixed-case">ET</jats:styled-content>) often suffer from neurological symptoms (<jats:styled-content style="fixed-case">NS</jats:styled-content>) not ever resulting from previous thrombotic cerebral events (<jats:styled-content style="fixed-case">TCE</jats:styled-content>). We reported <jats:styled-content style="fixed-case">NS</jats:styled-content> occurred in 282 patients, in order to identify the factors influencing <jats:styled-content style="fixed-case">ET</jats:styled-content>‐related <jats:styled-content style="fixed-case">NS</jats:styled-content> in the absence of <jats:styled-content style="fixed-case">TCE</jats:styled-content>, and the response to therapy. Overall, 116 of 282 patients (41%) presented <jats:styled-content style="fixed-case">NS</jats:styled-content>; 101 of them (87%) reported subjective transient and fluctuating <jats:styled-content style="fixed-case">NS</jats:styled-content>, without concurrent <jats:styled-content style="fixed-case">TCE</jats:styled-content>, which we defined as <jats:styled-content style="fixed-case">ET</jats:styled-content>‐related <jats:styled-content style="fixed-case">NS</jats:styled-content>, by frequency: cephalalgia, chronic paresthesias, dizziness or hypotension, visual disturbances, and tinnitus. In univariate analysis, <jats:styled-content style="fixed-case">ET</jats:styled-content>‐related <jats:styled-content style="fixed-case">NS</jats:styled-content> resulted more frequently in young people (<jats:italic>P</jats:italic> = 0.017) and in females (<jats:italic>P</jats:italic> = 0.025). We found a higher prevalence of <jats:styled-content style="fixed-case">JAK</jats:styled-content>2V617F mutation in <jats:styled-content style="fixed-case">ET</jats:styled-content>‐related <jats:styled-content style="fixed-case">NS</jats:styled-content> patients (<jats:italic>P</jats:italic> = 0.021). In multivariate analysis, gender (<jats:italic>P</jats:italic> = 0.024) and <jats:styled-content style="fixed-case">JAK</jats:styled-content>2<jats:styled-content style="fixed-case">V</jats:styled-content>617<jats:styled-content style="fixed-case">F</jats:styled-content> mutation (<jats:italic>P</jats:italic> = 0.041) remained significantly associated with the development of <jats:styled-content style="fixed-case">ET</jats:styled-content>‐related <jats:styled-content style="fixed-case">NS</jats:styled-content>, with a risk of about four times higher for <jats:styled-content style="fixed-case">JAK</jats:styled-content>2<jats:styled-content style="fixed-case">V</jats:styled-content>617<jats:styled-content style="fixed-case">F</jats:styled-content>‐mutated patients (<jats:styled-content style="fixed-case">OR</jats:styled-content> = 3.75). Ninety‐seven of 101 patients with <jats:styled-content style="fixed-case">ET</jats:styled-content>‐related <jats:styled-content style="fixed-case">NS</jats:styled-content> received an antiplatelet (<jats:styled-content style="fixed-case">AP</jats:styled-content>) agent at the time of <jats:styled-content style="fixed-case">NS</jats:styled-content>, whereas only selected high‐risk <jats:styled-content style="fixed-case">ET</jats:styled-content>‐related <jats:styled-content style="fixed-case">NS</jats:styled-content> patients were treated with a cytoreductive drug, according to the published guidelines and similarly to patients without <jats:styled-content style="fixed-case">NS</jats:styled-content>. We observed that only 32 of 97 (33%) patients with <jats:styled-content style="fixed-case">ET</jats:styled-content>‐related <jats:styled-content style="fixed-case">NS</jats:styled-content> achieved a complete response after <jats:styled-content style="fixed-case">AP</jats:styled-content> treatment. Among the 65 non‐responder patients, 36 (55.4%) improved <jats:styled-content style="fixed-case">NS</jats:styled-content> after the introduction of cytoreductive therapy; therefore, the addition of cytoreductive treatment should be considered in this setting.</jats:p>