Emir C Roach1, Margaret M Park2, W H Wilson Tang2, James D Thomas2, Kewal Asosingh1, Matt Kalaycio3, Serpil C Erzurum4, Samar Farha5. 1. Department of Pathobiology, Lerner Research Institute. 2. Heart & Vascular Institute. 3. Taussig Cancer Institute. 4. Department of Pathobiology, Lerner Research Institute; Respiratory Institute, Cleveland Clinic, Cleveland, Ohio. 5. Department of Pathobiology, Lerner Research Institute; Respiratory Institute, Cleveland Clinic, Cleveland, Ohio. Electronic address: farhas@ccf.org.
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.
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: Y Kunisaki; K Takase; T Miyamoto; M Fukata; A Nonami; K Kamezaki; Y Kaji; H Gondo; M Harada; K Nagafuji Journal: Bone Marrow Transplant Date: 2007-07-09 Impact factor: 5.483
Authors: A Cortelezzi; G Gritti; N Del Papa; M C Pasquini; R Calori; U Gianelli; M Cortiana; G Parati; F Onida; F Sozzi; C Vener; P Bianchi; G L Deliliers Journal: Leukemia Date: 2007-09-13 Impact factor: 11.528
Authors: Kewal Asosingh; Samar Farha; Alan Lichtin; Brian Graham; Deepa George; Micheala Aldred; Stanley L Hazen; James Loyd; Rubin Tuder; Serpil C Erzurum Journal: Blood Date: 2012-06-28 Impact factor: 22.113
Authors: Samar Farha; Kewal Asosingh; Weiling Xu; Jacqueline Sharp; Deepa George; Suzy Comhair; Margaret Park; W H Wilson Tang; James E Loyd; Karl Theil; Raymond Tubbs; Eric Hsi; Alan Lichtin; Serpil C Erzurum Journal: Blood Date: 2011-01-21 Impact factor: 22.113
Authors: Kewal Asosingh; Micheala A Aldred; Amit Vasanji; Judith Drazba; Jacqueline Sharp; Carol Farver; Suzy A A Comhair; Weiling Xu; Lauren Licina; Lan Huang; Bela Anand-Apte; Mervin C Yoder; Rubin M Tuder; Serpil C Erzurum Journal: Am J Pathol Date: 2008-02-07 Impact factor: 4.307