New Onset of Myelofibrosis in Association with Pulmonary Arterial Hypertension

Letters20 September 2005New Onset of Myelofibrosis in Association with Pulmonary Arterial HypertensionUday Popat, MD, Adaani Frost, MD, Enli Liu, MD, Romelia May, Remzi Bag, MD, Vishnu Reddy, MD, and Josef T. Prchal, MDUday Popat, MDFrom Baylor College of Medicine and The Methodist Hospital, Houston, TX 77030, and University of Alabama at Birmingham, Birmingham, AL 35233.Search for more papers by this author, Adaani Frost, MDFrom Baylor College of Medicine and The Methodist Hospital, Houston, TX 77030, and University of Alabama at Birmingham, Birmingham, AL 35233.Search for more papers by this author, Enli Liu, MDFrom Baylor College of Medicine and The Methodist Hospital, Houston, TX 77030, and University of Alabama at Birmingham, Birmingham, AL 35233.Search for more papers by this author, Romelia MayFrom Baylor College of Medicine and The Methodist Hospital, Houston, TX 77030, and University of Alabama at Birmingham, Birmingham, AL 35233.Search for more papers by this author, Remzi Bag, MDFrom Baylor College of Medicine and The Methodist Hospital, Houston, TX 77030, and University of Alabama at Birmingham, Birmingham, AL 35233.Search for more papers by this author, Vishnu Reddy, MDFrom Baylor College of Medicine and The Methodist Hospital, Houston, TX 77030, and University of Alabama at Birmingham, Birmingham, AL 35233.Search for more papers by this author, and Josef T. Prchal, MDFrom Baylor College of Medicine and The Methodist Hospital, Houston, TX 77030, and University of Alabama at Birmingham, Birmingham, AL 35233.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-143-6-200509200-00017 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail TO THE EDITOR:Background: Marrow fibrosis (myelofibrosis) occurs in patients with idiopathic myelofibrosis and other myeloproliferative disorders, which are characterized by a clonal hematopoiesis (1), and in patients with lupus erythematosus and cancer involving marrow. However, to our knowledge, myelofibrosis has not been previously reported in patients presenting with pulmonary arterial hypertension. The observation of severe myelofibrosis in a patient with this disorder prompted us to systematically evaluate patients with pulmonary arterial hypertension and some evidence of hematologic abnormality (for example, thrombocytopenia or anemia). Clonal proliferation of endothelial cells has been shown to occur in idiopathic pulmonary arterial hypertension (2). ...References1. Tefferi A. Myelofibrosis with myeloid metaplasia. N Engl J Med. 2000;342:1255-65. [PMID: 10781623] CrossrefMedlineGoogle Scholar2. Lee SD, Shroyer KR, Markham NE, Cool CD, Voelkel NF, Tuder RM. Monoclonal endothelial cell proliferation is present in primary but not secondary pulmonary hypertension. J Clin Invest. 1998;101:927-34. [PMID: 9486960] CrossrefMedlineGoogle Scholar3. Jacobson RJ, Salo A, Fialkow PJ. Agnogenic myeloid metaplasia: a clonal proliferation of hematopoietic stem cells with secondary myelofibrosis. Blood. 1978;51:189-94. [PMID: 620081] CrossrefMedlineGoogle Scholar4. Liu E, Jelinek J, Pastore YD, Guan Y, Prchal JF, Prchal JT. Discrimination of polycythemias and thrombocytoses by novel, simple, accurate clonality assays and comparison with PRV-1 expression and BFU-E response to erythropoietin. Blood. 2003;101:3294-301. [PMID: 12515724] CrossrefMedlineGoogle Scholar5. Prchal JT, Prchal JF, Belickova M, Chen S, Guan Y, Gartland GL, et al. Clonal stability of blood cell lineages indicated by X-chromosomal transcriptional polymorphism. J Exp Med. 1996;183:561-7. [PMID: 8627167] CrossrefMedlineGoogle Scholar Author, Article, and Disclosure InformationAuthors: Uday Popat, MD; Adaani Frost, MD; Enli Liu, MD; Romelia May; Remzi Bag, MD; Vishnu Reddy, MD; Josef T. Prchal, MDAffiliations: From Baylor College of Medicine and The Methodist Hospital, Houston, TX 77030, and University of Alabama at Birmingham, Birmingham, AL 35233.Note: Drs. Popat and Frost contributed equally to this study and should both be considered first authors. Dr. Prchal is also affiliated with School of Medicine, Charles University, Prague, Czech Republic.Grant Support: By a grant from the National Heart, Lung, and Blood Institute (R01HL5007-10 NHLBI) to Dr. Prchal.Disclosures: None disclosed. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byPulmonary hypertension is associated with poor cardiovascular and hematologic outcomes in patients with myeloproliferative neoplasms and cardiovascular diseaseEvaluation of hemodynamic parameters among patients with myeloproliferative neoplasms and suspected pulmonary hypertensionSevere but reversible pulmonary hypertension in scleromyxedema and multiple myeloma: a case reportThe hallmarks of severe pulmonary arterial hypertension: the cancer hypothesis—ten years laterPrevalence of pulmonary hypertension in myelofibrosisProtective role of myelocytic nitric oxide synthases in pulmonary hypertension due to lung diseases and/or hypoxiaINFECTION AND REACTIVE CHANGESEndothelial to haematopoietic transition contributes to pulmonary arterial hypertensionResolution of Myelofibrosis‐Associated Pulmonary Arterial Hypertension following Allogeneic Hematopoietic Stem Cell TransplantationBone Marrow–derived Cells Contribute to the Pathogenesis of Pulmonary Arterial HypertensionFibrogenesis in Primary Myelofibrosis: Diagnostic, Clinical, and Therapeutic ImplicationsImpaired right ventricular-pulmonary vascular function in myeloproliferative neoplasmsPulmonary Hypertension Associated with Chronic Hemolytic Anemia and Other Blood DisordersPulmonary vascular disease in mice xenografted with human BM progenitors from patients with pulmonary arterial hypertensionPlatelets in Pulmonary Vascular Physiology and PathologyHypoxia-inducible factors in human pulmonary arterial hypertension: a link to the intrinsic myeloid abnormalitiesPlatelets in Pulmonary Vascular Physiology and PathologyPulmonary Arterial Hypertension Related to Gaucher’s Disease, Sarcoidosis, and Other DisordersThe lung megakaryocytes and pulmonary fibrosis in systemic sclerosisBone marrow fibrosis: pathophysiology and clinical significance of increased bone marrow stromal fibresDepletion of Endothelial Progenitor Cells May Link Pulmonary Fibrosis and Pulmonary HypertensionHigh levels of circulating CD34 cells, dacrocytes, clonal hematopoiesis, and JAK2 mutation differentiate myelofibrosis with myeloid metaplasia from secondary myelofibrosis associated with pulmonary hypertensionChronic Myeloproliferative Disorders—Introduction 20 September 2005Volume 143, Issue 6 Page: 466-467 Keywords Blood Bone marrow Connective tissue diseases Fibrosis Granulocytes Hematopoiesis Lupus erythematosus Myeloproliferative disorders Platelets Pulmonary hypertension ePublished: 20 September 2005 Issue Published: 20 September 2005 Copyright & PermissionsCopyright © 2005 by American College of Physicians. All Rights Reserved.PDF downloadLoading ...