Pulmonary Capillary Hemangiomatosis: Report of a Case and Review of the Literature

Domingo, Christian; Encabo, B; Roig, Jordi; López, Dolores; Morera, Josep

doi:10.1159/000196053

Cited by 30 publications

(23 citation statements)

References 8 publications

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“…Secondary thromboembolism appears to be one of the factors responsible for the pulmonary hypertension in PCH. Thromboemboli, mostly in pulmonary arteries, were observed in some [4,5,16] but not all cases of PCH [2,5,8,11,12,14,19,20]. Combining the previously reported data, thromboembolism may not play a major role in the development of pulmonary hypertension in PCH.…”

Section: Discussionmentioning

confidence: 51%

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An autopsy case of pulmonary capillary hemangiomatosis without evidence of pulmonary hypertension

et al. 2001

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We report a case of pulmonary capillary hemangiomatosis (PCH) affecting a 37-year-old woman. PCH is a rare disease accompanying intrapulmonary capillary growth and pulmonary hypertension. In the present case, capillaries infiltrated the alveolar and bronchial walls but not the vascular walls. No signs or symptoms of pulmonary hypertension or right heart hypertrophy/dilatation were present. These findings suggest that vascular involvement in PCH is responsible for the induction of pulmonary hypertension.

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Section: Discussionmentioning

confidence: 51%

“…Pulmonary capillary hemangiomatosis is a recently described rare condition [22] and less than 30 cases have been reported in the literature [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24]. Age, sex, symptoms, pulmonary artery pressure, and pathological findings of available cases are listed in Table 1.…”

Section: Discussionmentioning

confidence: 99%

An autopsy case of pulmonary capillary hemangiomatosis without evidence of pulmonary hypertension

et al. 2001

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“…In this condition, new capillary-sized, thin-walled blood vessels grow in the lung interstitium and in and around pulmonary blood vessels and airways. Pulmonary capillary haemangiomatosis is associated with pulmonary hypertension and pulmonary veno-occlusive disease [21], and has been described after radiation therapy and anti-oestrogen therapy in a patient with breast cancer [22]. On light microscopic sections ( fig.…”

Section: Discussionmentioning

confidence: 99%

Human alveolar capillaries undergo angiogenesis in pulmonary veno-occlusive disease

Schraufnagel

Sekosan²,

McGee³

et al. 1996

Eur Respir J

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H Hu um ma an n a al lv ve eo ol la ar r c ca ap pi il ll la ar ri ie es s u un nd de er rg go o a an ng gi io og ge en ne es si is s i in n p pu ul lm mo on na ar ry y v ve en no o--o oc cc cl lu us si iv ve e d di is se ea as se e ABSTRACT: The bronchial circulation undergoes angiogenesis in several pathological conditions, such as lung neoplasm and bronchiectasis, but whether the pulmonary circulation can do this has been questioned. A woman treated with mitomycin C and 5-fluorouracil developed progressive, fatal pulmonary hypertension over 5 months. In addition to light and transmission electron microscopic examination of her lung, her pulmonary vasculature was cast and the casts were studied with scanning electron microscopy.Light microscopy showed that she had pulmonary veno-occlusive disease and angiomatoid capillary growth in the alveolar walls. Transmission electron microscopy confirmed the presence of pulmonary hypertension and showed thickened endothelial basement membrane. Scanning electron microscopy of the cast blood vessels showed distortion and destruction of alveolar capillaries prohibiting the passage of erythrocytes. Large new capillaries developed on top of, and were connected to, the shrivelled capillaries that made up the alveolar wall. The new capillaries were larger and fewer, which reduced the alveolar-capillary interface. Arteries and veins were irregularly narrowed and the veins had broad muscularity. Oedema was present, and the pulmonary lymphatics were extensively cast, especially in the lobular septa, but the lymphatics had a normal appearance.It appears that this patient suffered extensive capillary damage and venous occlusion and that the response was extensive new capillary formation, sometimes in angiomatoid configurations, and hypertrophy of pulmonary veins and arteries. Casting the microvasculature and viewing it with scanning electron microscopy identified new alveolar capillaries in this patient with acquired pulmonary hypertension.

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“…Mediastinal and hilar lymphadenomegaly has also been recognised as frequent (three in nine cases) [6] and even as a very suggestive radiological feature (12/15 cases) of PVOD [13]. Oedema and sinus histiocytosis [14], congestion and capillary-sized blood vessels [7], capillary proliferation [3] and vascular transformation of sinuses [4] have only been reported in single cases, but no study has specifically dealt with lymph nodes pathology in PVOD and PCH. Therefore, we undertook a review of a series of pulmonary and mediastinal lymph nodes resected with native lungs during transplantation for PVOD and PCH in our institution.…”

Section: Introductionmentioning

confidence: 99%

Lymph node pathology in pulmonary veno-occlusive disease and pulmonary capillary heamangiomatosis

et al. 2008

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To assess the histological bases of lymphadenomegaly, which has been reported as a frequent radiological finding in pulmonary veno-occlusive disease (PVOD), we have reviewed pulmonary and mediastinal lymph nodes resected during lung transplantations in 19 patients suffering from PVOD and related pulmonary capillary haemangiomatosis (PCH). Lymphatic congestion was common and was often obvious in subsegmental and segmental lymph nodes. Vascular transformation of the sinuses, intra-sinusal haemorrhage with erythrophagocytosis and lymphoid follicular hyperplasia were frequent especially in lobar, hilar and mediastinal lymph nodes. These lesions were very significantly less frequent in 33 cases of pulmonary hypertension unrelated to PVOD. Due to their thoracic location, these non-specific lesions could simulate other diagnoses such as Castleman disease or lymphangioleiomyomatosis. However, in the setting of pulmonary hypertension, they should suggest PVOD and PCH. They are probably secondary to venous congestion, veno-lymphatic shunts and angiogenetic factors associated with these diseases.

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Pulmonary Capillary Hemangiomatosis: Report of a Case and Review of the Literature

Cited by 30 publications

References 8 publications

An autopsy case of pulmonary capillary hemangiomatosis without evidence of pulmonary hypertension

An autopsy case of pulmonary capillary hemangiomatosis without evidence of pulmonary hypertension

Human alveolar capillaries undergo angiogenesis in pulmonary veno-occlusive disease

Lymph node pathology in pulmonary veno-occlusive disease and pulmonary capillary heamangiomatosis

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