Pulmonary Capillary Hemangiomatosis: A Rare Cause of Pulmonary Hypertension
| Autor: | Miriam D. Post, Mary C. O'Keefe |
|---|---|
| Rok vydání: | 2015 |
| Předmět: |
Chest Pain
Pathology medicine.medical_specialty Lung Neoplasms Hypertension Pulmonary medicine.medical_treatment Prostacyclin Pulmonary capillary hemangiomatosis Chest pain Pathology and Forensic Medicine Diagnosis Differential Hemangioma medicine Humans Lung transplantation Hemangioma Capillary Lung Fatigue Respiratory distress business.industry Venoocclusive disease General Medicine Prognosis medicine.disease Pulmonary hypertension Capillaries Medical Laboratory Technology Dyspnea Cough medicine.symptom business medicine.drug |
| Zdroj: | Archives of Pathology & Laboratory Medicine. 139:274-277 |
| ISSN: | 1543-2165 0003-9985 |
| DOI: | 10.5858/arpa.2013-0500-rs |
| Popis: | Pulmonary capillary hemangiomatosis is a rare cause of pulmonary hypertension characterized by extensive proliferation of pulmonary capillaries within alveolar septae. Clinical presentation is nonspecific and includes dyspnea, cough, chest pain, and fatigue. Radiology shows diffuse centrilobular ground-glass opacities. Pulmonary capillary hemangiomatosis is clinically and radiographically indistinguishable from peripheral venoocclusive disease, making microscopic diagnosis essential. Histologically, pulmonary capillary hemangiomatosis shows an abnormal proliferation of small, thin-walled capillaries that expand the alveolar septae. The endothelial cells that comprise these lesions are cytologically bland and show no mitotic activity. Pulmonary capillary hemangiomatosis is important to recognize because prostacyclin therapy, a mainstay in the treatment of pulmonary hypertension, has been reported to cause sudden respiratory distress and death in these patients. Prognosis of this disease remains poor, and the only definitive treatment is lung transplantation. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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