Chest
Original Research: Pulmonary HypertensionPlatelet-Derived Growth Factor Is Increased in Pulmonary Capillary Hemangiomatosis
Section snippets
Materials and Methods
We obtained lung tissue samples from two patients with PCH and normal lung tissue from seven patients who had undergone lobectomies performed for neoplastic disease. These control subjects were similar to those in the single published microarray study of PAH.10
Results
High-power histologic examination showed that the alveolar septa exhibited proliferations of capillary-sized vessels on both sides of the alveolar wall, giving a nodular appearance (Fig 1). Small and large muscular pulmonary arteries showed hypertensive changes, including intimal and medial thickening without plexiform lesions.
Unsupervised clustering performed using a list of 7,567 genes filtered for low expression showed two groups within the data set, corresponding to the PCH case patients
Discussion
PCH has been reclassified as a form of PAH associated with significant venous or capillary involvement.11PCH has a peak incidence in the second and third decade, affecting men and women equally.2Most cases appear sporadic, with one report of familial occurrence.12Imaging with chest radiography and CT scanning shows nodular pulmonary infiltrates, septal lines, lymphadenopathy, and occasional pleural effusions, distinguishing PCH from other forms of PAH13, 14; however, a definitive diagnosis can
References (35)
- et al.
Pulmonary capillary hemangiomatosis
Hum Pathol
(1986) - et al.
Pulmonary capillary hemangiomatosis with atypical endotheliomatosis: successful antiangiogenic therapy with doxycycline
Chest
(2003) - et al.
Endothelial nitric oxide synthase expression in pulmonary capillary hemangiomatosis
Exp Mol Pathol
(2005) - et al.
Pulmonary capillary hemangiomatosis arising in hereditary hemorrhagic telangiectasia
Hum Pathol
(2004) - et al.
Abnormalities in pericytes on blood vessels and endothelial sprouts in tumors
Am J Pathol
(2002) - et al.
Capillary haemangiomatosis of the lungs
Histopathology
(1978) - et al.
Pulmonary capillary hemangiomatosis associated with primary pulmonary hypertension: report of 2 new cases and review of 35 cases from the literature
Medicine (Baltimore)
(2002) - et al.
Pathologic assessment of vasculopathies in pulmonary hypertension
J Am Coll Cardiol
(2004) - et al.
Pulmonary capillary hemangiomatosis: an immunohistochemical analysis of vascular remodeling
Eur J Med Res
(2006) - et al.
Pulmonary capillary hemangiomatosis: results of gene expression analysis
Chest
(2005)
Gene expression patterns in the lungs of patients with primary pulmonary hypertension: a gene microarray analysis
Circ Res
Clinical classification of pulmonary hypertension
J Am Coll Cardiol
Familial pulmonary capillary hemangiomatosis resulting in primary pulmonary hypertension
Ann Intern Med
High-resolution CT of the chest in four patients with pulmonary capillary hemangiomatosis or pulmonary venoocclusive disease
AJR Am J Roentgenol
Pulmonary capillary hemangiomatosis: radiographic appearance
J Thorac Imaging
Treatment of pulmonary hemangiomatosis with recombinant interferon α-2a
N Engl J Med
Pericyte loss and microaneurysm formation in PDGF-B-deficient mice
Science
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This research was supported in part by the Department of Pathology, Columbia University College of Physicians and Surgeons, and National Institutes of Health grant HL67771.
The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.
Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/misc/reprints.shtml).