title: Impaired right ventricular-pulmonary vascular function in myeloproliferative neoplasms.
abstract: BACKGROUND
  Increased bone marrow hemangioblast numbers, alterations in erythroid/myeloid lineages, increased reticulin, and greater circulating bone marrow progenitor cells are present in patients with pulmonary arterial hypertension (PAH). The data suggest that myeloid progenitors contribute to the pathogenesis of PAH, but there are little data on the prevalence of pulmonary vascular disease among the different forms of myeloid diseases. We hypothesized that there would be a higher prevalence of pulmonary vascular disease in myeloproliferative neoplasms that have high circulating progenitor cells, such as myelofibrosis and chronic myelogenous leukemia (CML), compared with those with low circulating progenitors, such as in aplastic anemia.
  
  
  METHODS
  Patients with myelofibrosis, CML, and aplastic anemia who underwent echocardiographic evaluation of cardiac function in preparation for bone marrow transplantation at the Cleveland Clinic between 1997 and 2012 were identified and their electronic medical records were queried for demographic data, blood cell counts, and pulmonary function tests. All echocardiograms were uniformly analyzed in a blinded fashion by an advanced sonographer and cardiologist for measures of right and left ventricular function and estimation of pulmonary vascular disease.
  
  
  RESULTS
  Gender and race distribution among disease groups was similar. Patients with myelofibrosis (n = 19) and aplastic anemia (n = 30) had increased right ventricle (RV) wall thickness compared with CML (n = 82) patients (aplastic anemia, 0.7 ± 0.1; CML, 0.5 ± 0.1; and myelofibrosis, 0.7 ± 0.1; p = 0.02). Patients with myelofibrosis had higher levels of estimated RV systolic pressure compared with the other groups (aplastic anemia, 29.9 ± 1.5; CML, 26.2 ± 1.1; and myelofibrosis, 36.7 ± 3.7 mm Hg; p < 0.01).
  
  
  CONCLUSIONS
  The findings suggest an important role for myeloid progenitors in the maintenance of pulmonary-vascular health, in which abnormal myeloproliferative progenitors are associated with RV pathology.
authors: Department of Pathobiology, Lerner Research Institute.
authors: Emir C
authors: EC
authors: Roach
authors: Heart & Vascular Institute.
authors: Margaret M
authors: MM
authors: Park
authors: Heart & Vascular Institute.
authors: W H Wilson
authors: WH
authors: Tang
authors: Heart & Vascular Institute.
authors: James D
authors: JD
authors: Thomas
authors: Department of Pathobiology, Lerner Research Institute.
authors: Kewal
authors: K
authors: Asosingh
authors: Taussig Cancer Institute.
authors: Matt
authors: M
authors: Kalaycio
authors: Department of Pathobiology, Lerner Research Institute; Respiratory Institute, Cleveland Clinic, Cleveland, Ohio.
authors: Serpil C
authors: SC
authors: Erzurum
authors: Department of Pathobiology, Lerner Research Institute; Respiratory Institute, Cleveland Clinic, Cleveland, Ohio. Electronic address: farhas@ccf.org.
authors: Samar
authors: S
authors: Farha
mesh_terms: D000328:Adult; D016026:Bone Marrow Transplantation; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009196:Myeloproliferative Disorders / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D011379:Prognosis; D011652:Pulmonary Circulation; D012189:Retrospective Studies; D014655:Vascular Resistance / Q000502:physiology*; D018497:Ventricular Dysfunction, Right / Q000209:etiology* / Q000503:physiopathology; D016278:Ventricular Function, Right / Q000502:physiology*
keywords: chronic myelogenous leukemia; myeloid progenitors; pulmonary arterial hypertension; pulmonary vascular disease
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