Long-term Effects of the Janus Kinase 1/2 Inhibitor Ruxolitinib on Pulmonary Hypertension and the Cardiac Function in a Patient with Myelofibrosis

  • Miyawaki Hiroshi
    Department of Cardiovascular Medicine, Osaka University Graduate School of Medicine, Japan
  • Kioka Hidetaka
    Department of Cardiovascular Medicine, Osaka University Graduate School of Medicine, Japan
  • Sato Kazuaki
    Department of Pathology, Osaka University Graduate School of Medicine, Japan
  • Kurashige Masako
    Department of Pathology, Osaka University Graduate School of Medicine, Japan
  • Ozawa Takayuki
    Department of Hematology and Oncology, Osaka University Graduate School of Medicine, Japan
  • Shibayama Hirohiko
    Department of Hematology and Oncology, Osaka University Graduate School of Medicine, Japan
  • Hikoso Shungo
    Department of Cardiovascular Medicine, Osaka University Graduate School of Medicine, Japan
  • Morii Eiichi
    Department of Pathology, Osaka University Graduate School of Medicine, Japan
  • Yamauchi-Takihara Keiko
    Department of Cardiovascular Medicine, Osaka University Graduate School of Medicine, Japan Health Care Center, Osaka University, Japan
  • Sakata Yasushi
    Department of Cardiovascular Medicine, Osaka University Graduate School of Medicine, Japan

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Description

<p>Constitutive activation of the Janus kinase/signal transduction and activator of transcription (JAK-STAT) signaling pathway plays a central role in the pathogenesis of myelofibrosis (MF) and pulmonary hypertension (PH) is a known complication of MF. On the other hand, it has been proposed that the JAK-STAT pathway, especially signal transducer and activation of transcription (STAT) 3 activation, protects cardiomyocytes from various stresses. We describe the case of a patient with MF-associated PH who developed left ventricular dysfunction after five years of treatment with the JAK 1/2 inhibitor, ruxolitinib. This is the first report with histopathological findings that demonstrate possible contradictory effects of a JAK 1/2 inhibitor: improvement of MF-associated PH and cardiotoxicity. </p>

Journal

  • Internal Medicine

    Internal Medicine 59 (2), 229-233, 2020-01-15

    The Japanese Society of Internal Medicine

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