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Imatinib as Rescue Therapy in a Patient With Pulmonary Hypertension Associated With Gaucher DiseaseImatinib for Pulmonary Hypertension in Gaucher

Nadine Al-Naamani, MD; Kari E. Roberts, MD; Nicholas S. Hill, MD, FCCP; Ioana R. Preston, MD, FCCP
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From the Department of Medicine, Pulmonary, Critical Care and Sleep Division, Tufts Medical Center, Boston, MA.

CORRESPONDENCE TO: Ioana R. Preston, MD, FCCP, Department of Medicine, Pulmonary, Critical Care and Sleep Division, Tufts Medical Center, 800 Washington St, Mailbox 257, Boston, MA 02111; e-mail: ipreston@tuftsmedicalcenter.org.


This article was presented in poster form at the 2014 American Thoracic Society Annual Meeting, May 20, 2014, San Diego, CA.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2014;146(3):e81-e83. doi:10.1378/chest.13-2795
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Pulmonary hypertension (PH) is a known complication of Gaucher disease (GD) and splenectomy. Although it resembles World Health Organization (WHO) group 1 pulmonary arterial hypertension (PAH), PH due to GD or splenectomy is part of WHO group 5. There are no clinical trials testing therapies in PH due to GD or splenectomy. Several reports suggest that PAH-specific therapies are beneficial in patients with PH due to GD, although data are insufficient to formulate a treatment algorithm for these patients. The tyrosine kinase inhibitor imatinib has been investigated in the treatment of severe PAH, but not in PH WHO group 5. We report a patient with GD and splenectomy who developed PH that progressed while on conventional PAH-specific therapy and improved once imatinib was added to her treatment regimen. This is the first report, to our knowledge, describing significant subjective and objective improvements in response to imatinib in a patient with WHO group 5 PH.

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