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Vol. 38. Issue 6.
Pages 291-294 (June 2002)
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Vol. 38. Issue 6.
Pages 291-294 (June 2002)
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Hemangiomatosis capilar pulmonar: una causa poco frecuente de hipertensión pulmonar
Pulmonary capillary hemangiomatosis: a rare cause of pulmonary hypertension
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R. Bandrés Gimeno
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rafaelbandres@yahoo.es

Correspondencia: Dr. Rafael Bandrés Gimeno Servicio de Neumología. Complexo Hospitalario Xeral-Cíes.Pizarro 22. 36204 Vigo. Pontevedra
, M.L. Torres Durán
Servicios de Neumología, Complexo Hospitalario Xeral-Cíes. Vigo.
J. González-Carreróa, B. Iglesiasa, C. Vicentea
a Servicios de Anatomía Patológica. Complexo Hospitalario Xeral-Cíes. Vigo
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La hemangiomatosis capilar pulmonar (HCP) es una cau-sa rara de hipertensión pulmonar, caracterizada por la proliferación de capilares que infiltran las estructuras del parénquima pulmonar, particularmente las vénulas, pero también los bronquiolos, el intersticio y otras. Presentamos un caso de hemangiomatosis capilar pulmonar en un varón de 70 años. Se demostró la existencia de una hipertensión arterial pulmonar mediante ecocardiografía y cateterismo. La tomografía axial computarizada (TAC) torácica evidenció que el enfermo presentaba un enfisema grave, a pesar de los valores de la espirometría que reflejaban una obstrucción al flujo aéreo de grado leve. Desarrolló una disnea progresiva y una insuficiencia respiratoria con efecto shunt marcado hasta el fallecimiento. El diagnóstico patológico se realizó en la autopsia. Se observó una proliferación capilar en las paredes alveolares, que formaba en ocasiones ovillos que protruían en los espacios aéreos o rodeaban pequeños vasos y bronquiolos. Las células endoteliales de los vasos neoformados no evidenciaban atipias y las mitosis eran esca-sas. La negatividad de la técnica inmunohistoquímica del antígeno p53 y la baja tasa de proliferación celular evaluada por Ki67 no apoyaban la naturaleza neoplásica de la HCP, como se ha sugerido en algunos trabajos

Palabras clave:
Hipertensión pulmonar
Hemangiomatosis capi-lar pulmonar

Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension characterized by capillary proliferation infiltrating the structures of the pulmonary parenchyma. Although veins are particularly involved, proliferation also affects bronchiolar, interstitial and other structures. We report a case of PCH in a 70-year-old man. Pulmonary artery hypertension was demonstrated by echocardiogram and angiography. Severe emphysema could be seen in a computed tomographic scan of the thorax, even though spirometric values indicated that airflow obstruction was mild. Dyspnea and respiratory insufficiency progressed with marked shunting until death. Tissue inspection at the autopsy revealed capillary proliferation in the alveolar walls with occasional oviform protrusions into air spaces or around small vessels and bronchioles. Endothelial cells in newly formed vessels were not atypical and mitosis was scarce; p53 expression was negative and Ki67 proliferation slight, indicating that PCH is not a neoplastic process as has sometimes been suggested

Keywords:
Pulmonary hypertension
Pulmonary capillary he-mangiomatosis
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Copyright © 2002. Sociedad Española de Neumología y Cirugía Torácica
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