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Pulmonary Capillary Hemangiomatosis*: Results of Gene Expression Analysis FREE TO VIEW

Steven M. Kawut, MD, MS, FCCP; Adel M. Assaad, MD; Selim M. Arcasoy, MD, FCCP; Erika B. Rosenzweig, MD; Joshua R. Sonett, MD; Alain C. Borczuk, MD
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From the Departments of Medicine (Drs. Kawut and Arcasoy), Pathology (Drs. Assaad and Borczuk), Pediatrics (Dr. Rosenzweig), and Surgery (Dr. Sonett), College of Physicians and Surgeons, and the Department of Epidemiology (Dr. Kawut), Joseph L. Mailman School of Public Health, Columbia University, New York, NY.

Correspondence to: Steven M. Kawut, MD, MS, FCCP, Division of Pulmonary, Allergy, and Critical Care Medicine, PH 8E, Room 101, 622 W 168th St, New York, NY 10032; e-mail: sk2097@columbia.edu

Chest. 2005;128(6_suppl):575S-576S. doi:10.1378/chest.128.6_suppl.575S
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Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension. The etiology of this disease is unknown, and there is no available medical therapy. We report on the gene expression profile in patients with PCH compared to those with healthy lungs.

A 23-year-old woman and an 18-year-old man presented with severe pulmonary hypertension and subtle ground-glass lung opacities determined by CT scan. Open-lung biopsy in both cases showed PCH. Both patients subsequently underwent lung transplantation. We performed needle microdissection of nodules of PCH from cryostat sections, and then RNA extraction, probe preparation, and hybridization to oligonucleotide arrays (U95A; Affymetrix; Santa Clara, CA). We used unsupervised clustering in comparing PCH samples to the gene expression profile of healthy lung tissue.

Platelet-derived growth factor receptor (PDGFR)-β, platelet-derived growth factor (PDGF)-B, angiopoietin (Ang)-1, mast cell genes, and type II pneumocyte genes were highly expressed in the samples from PCH patients. Immunohistochemical staining for PDGFR-β localized to perivascular/myoid-type cells surrounding the proliferating capillaries. The expression of PDGF was localized to type II pneumocytes and mast cells by in situ hybridization. CD117 staining confirmed an abundance of mast cells in the lesions.

PCH is characterized by increased expression of PDGFR-β, PDGF-B, Ang-1, and other mast cell genes. Mast cells have previously been implicated in angiogenesis, and express both PDGF and PDGFR. Ang-1 expression is variable in other forms of pulmonary hypertension. The up-regulation of these genes may suggest a mechanism and potential therapies for PCH. The administration of a selective tyrosine kinase receptor inhibitor could elucidate the role of PDGFR-β in PCH.

Supported in part by National Institutes of Health grant HL67771 and the Florence and Herbert Irving Clinical Research Career Award.

Abbreviations: Ang = angiopoietin; PCH = pulmonary capillary hemangiomatosis; PDGF = platelet-derived growth factor; PDGFR = platelet-derived growth factor receptor




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