New Onset of Myelofibrosis in Association with Pulmonary Arterial Hypertension
Authors: Uday Popat, Adaani Frost, Enli Liu, Romelia May, Remzi Bag, Vishnu Reddy, Josef T. Prchal
Journal: Annals of Internal Medicine (2005)

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. 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