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Pulmonary arterial hypertension in primary myelofibrosis is common and associated with an altered angiogenic status

Leukemia volume 22, pages 646–649 (2008)Cite this article

Angiogenesis plays an important role in the pathogenesis of both pulmonary arterial hypertension (PAH) and chronic myeloproliferative disorders (CMPDs).1, 2 The WHO classification of pulmonary hypertension includes CMPDs as diseases associated with PAH development1 and, according to the literature, pulmonary hypertension affects between 13 and 48% of patients with myeloproliferative diseases. It has been suggested that this comorbidity has a multifactorial pathobiological origin, including the presence of pulmonary myeloid metaplasia, the obstruction of pulmonary vessels by circulating megakaryocytes, smooth muscle hyperplasia due to platelet-derived growth factor (PDGF), and thromboembolic events.3, 4 However, little is known about the origin of the association, and the possible pathogenetic link between CMPDs and PAH has not yet been studied.

We investigated this association in primary myelofibrosis (PMF) diagnosed on the basis of the WHO classification.5 The study group consisted of 36 patients being followed up at our institution: 22 with PMF, 7 with PMF developing from polycythemia vera and 7 with PMF progressing from essential thrombocythemia. Their clinical characteristics are summarized in Table 1. The control groups consisted of 10 healthy subjects (controls) and 10 patients with pulmonary hypertension due to other causes (S-PH).

Table 1 Clinical characteristics of the 36 PMF patients (pts)
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Authors and Affiliations

  1. Department of Medical Sciences, University of Milan, Milan, Italy

    A Cortelezzi, M C Pasquini, F Onida, C Vener & G L Deliliers

  2. Hematology 1—Bone Marrow Transplantation Unit, IRCCS Foundation ‘Policlinico Mangiagalli and Regina Elena’, Milan, Italy

    A Cortelezzi, G Gritti, R Calori, M Cortiana, F Onida & G L Deliliers

  3. Department of Rheumatology, G. Pini Hospital, Milan, Italy

    N Del Papa

  4. Department of Pathology, University of Milan and San Paolo Hospital, Milan, Italy

    U Gianelli

  5. Department of Clinical Medicine, Prevention and Applied Biotechnologies, University of Milano-Bicocca and ‘Istituto Auxologico Italiano’, Milan, Italy

    G Parati

  6. Cardiology Unit, IRCCS Foundation ‘Policlinico Mangiagalli and Regina Elena’, Milan, Italy

    F Sozzi

  7. Hematology 2 Unit, IRCCS Foundation ‘Policlinico Mangiagalli and Regina Elena’, Milan, Italy

    P Bianchi

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  1. A Cortelezzi
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  2. G Gritti
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Correspondence to A Cortelezzi.

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Cortelezzi, A., Gritti, G., Del Papa, N. et al. Pulmonary arterial hypertension in primary myelofibrosis is common and associated with an altered angiogenic status. Leukemia 22, 646–649 (2008). https://doi.org/10.1038/sj.leu.2404943

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