Pulmonary capillary hemangiomatosis: a lesson learned
Autops. Case Rep
;
9(3): e2019111, July-Sept. 2019. ilus
Artículo
en Inglés
| LILACS
| ID: biblio-1016910
ABSTRACT
Pulmonary capillary hemangiomatosis (PCH) is a rare and controversial entity that is known to be a cause of pulmonary hypertension and is microscopically characterized by proliferation of dilated capillary-sized channels along and in the alveolar walls. Clinically, it is mostly seen in adults. Clinical features are characterized by nonspecific findings such as shortness of breath, cough, chest pain, and fatigue. It can be clinically indistinguishable from pre-capillary pulmonary arterial hypertension disorders such as primary pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension. However, the diagnostic distinction, which usually requires a multidisciplinary approach, is crucial in order to avoid inappropriate treatment with vasodilator medications usually used for PAH treatment. Prognosis of PCH remains poor with lung transplant being the only definitive treatment. We report an autopsy case of pulmonary capillary hemangiomatosis unmasked at autopsy that was treated with a prostacyclin analog, usually contraindicated in such patients. We emphasize that this entity should always be on the differential diagnosis in a patient with pulmonary hypertension and requires great vigilance on the part of the clinician, radiologist and pathologist to make the diagnosis and guide appropriate management.
Texto completo:
Disponible
Índice:
LILACS (Américas)
Asunto principal:
Hemangioma Capilar
Tipo de estudio:
Estudio diagnóstico
/
Estudio pronóstico
Límite:
Anciano
/
Femenino
/
Humanos
Idioma:
Inglés
Revista:
Autops. Case Rep
Asunto de la revista:
Anatomia
/
Patologia Cl¡nica
/
Patologia Legal
Año:
2019
Tipo del documento:
Artículo
País de afiliación:
Israel
/
Pakistán
/
Estados Unidos
Institución/País de afiliación:
Cedars Sinai Medical Center/US
/
Foundation University Medical College/PK
/
Tel Aviv University/IL
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