Familial Pulmonary Capillary Hemangiomatosis Early in Life
Figure 2
Lung biopsies of patient 1 (a and b), patient 2 (c and d), patient 3 (e), and control (f). (a) Altered lung architecture with slight emphysema and slight to moderate thickening of alveolar walls due to proliferation of capillaries. Note venous sclerosis (→) and the capillary duplications in some alveolar walls. Changes reflect pulmonary arterial hypertension (Hematoxylin and Eosin, ×100), (b) High-power view of thickened alveolar septa due to proliferation and tortuosity of capillaries. Endothelial cells lining the inner surface of capillaries are marked by CD-34 staining (Immunopoeroxidase, ×200), (c) Irregular emphysematous changes and focal capillary proliferation in thickend alveolar walls. Changes are less prominent than in patient 1 (Hematoxylin and Eosin, ×100), (d) Capillary proliferation and protrusion of tufts of capillaries is found in some alveolar septa (⇒) and in the wall of a vein (→). Endothelial cells lining the inner surface of capillaries are marked by CD-31 staining (Imminoperoxidase, ×200), (e and f) Post mortem lung histology of patient 3 (e) versus control (f). pseudoglandular stage of lung development at 15 weeks of gestational age. Endothelial cells express CD-31. (Immunoperoxidase, ×200), (e) Broadened space between pseudoglands with proliferation of capillaries in lung tissue of patient 3. Regressive changes are due to advanced autolysis following intrauterine death, (f) Normal lung architecture at 15 weeks of gestational age.
